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Sample records for manifestations rhumatologiques revelatrices

  1. Rheumatic manifestations of scurvy.

    PubMed

    Ferrari, Claudia; Possemato, Niccolò; Pipitone, Nicolò; Manger, Bernhard; Salvarani, Carlo

    2015-04-01

    This paper reviews the rheumatological manifestations of scurvy, based on articles published in English from 1965 until October 2014, with a particular focus on rheumatological manifestations. Scurvy is a rare, uncommon disease in developed countries. Due to its clinical heterogeneity, the disease can easily mimic rheumatologic conditions leading to a delay in diagnosis and treatment. PMID:25854491

  2. Rheumatic manifestations of scurvy.

    PubMed

    Ferrari, Claudia; Possemato, Niccolò; Pipitone, Nicolò; Manger, Bernhard; Salvarani, Carlo

    2015-04-01

    This paper reviews the rheumatological manifestations of scurvy, based on articles published in English from 1965 until October 2014, with a particular focus on rheumatological manifestations. Scurvy is a rare, uncommon disease in developed countries. Due to its clinical heterogeneity, the disease can easily mimic rheumatologic conditions leading to a delay in diagnosis and treatment.

  3. 40 CFR 262.21 - Manifest tracking numbers, manifest printing, and obtaining manifests.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... printing, and obtaining manifests. 262.21 Section 262.21 Protection of Environment ENVIRONMENTAL PROTECTION... Manifest § 262.21 Manifest tracking numbers, manifest printing, and obtaining manifests. (a)(1) A registrant may not print, or have printed, the manifest for use of distribution unless it has...

  4. Haematological manifestations of lupus

    PubMed Central

    Fayyaz, Anum; Igoe, Ann; Kurien, Biji T; Danda, Debashish; James, Judith A; Stafford, Haraldine A; Scofield, R Hal

    2015-01-01

    Our purpose was to compile information on the haematological manifestations of systemic lupus erythematosus (SLE), namely leucopenia, lymphopenia, thrombocytopenia, autoimmune haemolytic anaemia (AIHA), thrombotic thrombocytopenic purpura (TTP) and myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manifestation. Neutropenia can lead to morbidity and mortality from increased susceptibility to infection. Severe neutropenia can be successfully treated with granulocyte colony-stimulating factor. While related to disease activity, there is no specific therapy for lymphopenia. Severe lymphopenia may require the use of prophylactic therapy to prevent select opportunistic infections. Isolated idiopathic thrombocytopenic purpura maybe the first manifestation of SLE by months or even years. Some manifestations of lupus occur more frequently in association with low platelet count in these patients, for example, neuropsychiatric manifestation, haemolytic anaemia, the antiphospholipid syndrome and renal disease. Thrombocytopenia can be regarded as an important prognostic indicator of survival in patients with SLE. Medical, surgical and biological treatment modalities are reviewed for this manifestation. First-line therapy remains glucocorticoids. Through our review, we conclude glucocorticoids do produce a response in majority of patients initially, but sustained response to therapy is unlikely. Glucocorticoids are used as first-line therapy in patients with SLE with AIHA, but there is no conclusive evidence to guide second-line therapy. Rituximab is promising in refractory and non-responding AIHA. TTP is not recognised as a criteria for classification of SLE, but there is a considerable overlap between the presenting features of TTP and SLE, and a few patients with SLE have concurrent

  5. [Unusual Migraine Manifestations].

    PubMed

    Schipper, Sivan; Gantenbein, Andreas R; Sandor, Peter S

    2016-06-01

    Migraine is a complex neurologic disorder by which several systems of the central nervous system (autonomous system, affective, cognitive, sensoric and motoric system) may be affected on different levels. Around a fourth of the patients have migraine aura. The most common aura is the visual aura, followed by sensoric aura. But motoric deficits as well as deficits of higher cortical centers (disorders of thinking, orientation, coherence or concentration) may occur as well. In analogy with a headache calendar, an aura calendar can deliver important help in the diagnostic process of rare migraine manifestations and prevent underdiagnosis of unusual migraine manifestations. Complex migraine manifestations are diagnoses of exlusion, and a broad diagnostic work-up is warranted in order to exclude dangerous neurologic pathologies. There are no specific therapeutic recommendations, as there is a lack of randomized controlled studies.

  6. [Unusual Migraine Manifestations].

    PubMed

    Schipper, Sivan; Gantenbein, Andreas R; Sandor, Peter S

    2016-06-01

    Migraine is a complex neurologic disorder by which several systems of the central nervous system (autonomous system, affective, cognitive, sensoric and motoric system) may be affected on different levels. Around a fourth of the patients have migraine aura. The most common aura is the visual aura, followed by sensoric aura. But motoric deficits as well as deficits of higher cortical centers (disorders of thinking, orientation, coherence or concentration) may occur as well. In analogy with a headache calendar, an aura calendar can deliver important help in the diagnostic process of rare migraine manifestations and prevent underdiagnosis of unusual migraine manifestations. Complex migraine manifestations are diagnoses of exlusion, and a broad diagnostic work-up is warranted in order to exclude dangerous neurologic pathologies. There are no specific therapeutic recommendations, as there is a lack of randomized controlled studies. PMID:27269777

  7. Thermodynamics in 'Manifest Reality'

    SciTech Connect

    Hankey, Alex

    2010-12-22

    D'Espagnat's proof that the universe is not a 'strongly objective reality' demands that all physical processes are reconsidered in that light. D'Espagnat suggests a 'Veiled Reality' as a suitable alternative. The most economical way to achieve that is to demand that 'information production' at a quantum level creates the basis for self-consistent perception of a world of macroscopic, 'manifest' entities, as opposed to self-existent objects. Such a 'manifest reality' fulfils both Wheeler's attempt at an 'IT-from-BIT' programme, and Zeilinger's suggestion that 'information is primary'.

  8. Immunologic manifestations of autophagy

    PubMed Central

    Deretic, Vojo; Kimura, Tomonori; Timmins, Graham; Moseley, Pope; Chauhan, Santosh; Mandell, Michael

    2015-01-01

    The broad immunologic roles of autophagy span innate and adaptive immunity and are often manifested in inflammatory diseases. The immune effects of autophagy partially overlap with its roles in metabolism and cytoplasmic quality control but typically expand further afield to encompass unique immunologic adaptations. One of the best-appreciated manifestations of autophagy is protection against microbial invasion, but this is by no means limited to direct elimination of intracellular pathogens and includes a stratified array of nearly all principal immunologic processes. This Review summarizes the broad immunologic roles of autophagy. Furthermore, it uses the autophagic control of Mycobacterium tuberculosis as a paradigm to illustrate the breadth and complexity of the immune effects of autophagy. PMID:25654553

  9. [Respiratory manifestations in aspergillosis].

    PubMed

    Regimbaud, M

    1986-01-01

    Aspergillus is a genus of cosmopolitan fungi with a selective pulmonary tropism. Their pathogenic role is due either to spreading in pre-existing pulmonary cavities, or to their allergizing capacity. Cavitary sequellae of tuberculosis and suppuration, particularly frequent and important in tropical environment, are elective localization for Aspergillus colonization. Surgical treatment is nowadays the only efficient one. Allergic manifestations are a more complex problem of therapy, exclusion of allergen being difficult to get in tropical environment. PMID:3773680

  10. Neurological manifestations of malaria.

    PubMed

    Román, G C; Senanayake, N

    1992-03-01

    The involvement of the nervous system in malaria is reviewed in this paper. Cerebral malaria, the acute encephalopathy which complicates exclusively the infection by Plasmodium falciparum commonly affects children and adolescents in hyperendemic areas. Plugging of cerebral capillaries and venules by clumped, parasitized red cells causing sludging in the capillary circulation is one hypothesis to explain its pathogenesis. The other is a humoral hypothesis which proposes nonspecific, immune-mediated, inflammatory responses with release of vasoactive substances capable of producing endothelial damage and alterations of permeability. Cerebral malaria has a mortality rate up to 50%, and also a considerable longterm morbidity, particularly in children. Hypoglycemia, largely in patients treated with quinine, may complicate the cerebral symptomatology. Other central nervous manifestations of malaria include intracranial hemorrhage, cerebral arterial occlusion, and transient extrapyramidal and neuropsychiatric manifestations. A self-limiting, isolated cerebellar ataxia, presumably caused by immunological mechanisms, in patients recovering from falciparum malaria has been recognized in Sri Lanka. Malaria is a common cause of febrile seizures in the tropics, and it also contributes to the development of epilepsy in later life. Several reports of spinal cord and peripheral nerve involvement are also available. A transient muscle paralysis resembling periodic paralysis during febrile episodes of malaria has been described in some patients. The pathogenesis of these neurological manifestations remains unexplored, but offers excellent perspectives for research at a clinical as well as experimental level. PMID:1307475

  11. Ophthalmologic manifestations of celiac disease

    PubMed Central

    Martins, Thiago Gonçalves dos Santos; Costa, Ana Luiza Fontes de Azevedo; Oyamada, Maria Kiyoko; Schor, Paulo; Sipahi, Aytan Miranda

    2016-01-01

    Celiac disease is an autoimmune disorder that affects the small intestine of genetically predisposed individuals. Ophthalmic manifestations are within the extra-intestinal manifestations, and can be divided into those of autoimmune disorders or those due to absorptive disabilities. This article reviewed the ophthalmologic manifestation of celiac disease. Ophthalmic symptoms are rare, but should be investigated in patients with celiac disease and taken into consideration as the first systemic manifestation. PMID:26949627

  12. Rheumatologic manifestations of sarcoidosis.

    PubMed

    Sweiss, Nadera J; Patterson, Karen; Sawaqed, Ray; Jabbar, Umair; Korsten, Peter; Hogarth, Kyle; Wollman, Robert; Garcia, Joe G N; Niewold, Timothy B; Baughman, Robert P

    2010-08-01

    Sarcoidosis is a systemic, clinically heterogeneous disease characterized by the development of granulomas. Any organ system can be involved, and patients may present with any number of rheumatologic symptoms. There are no U.S. Food and Drug Administration-approved therapies for the treatment of sarcoidosis. Diagnosing sarcoidosis becomes challenging, particularly when its complications cause patients' symptoms to mimic other conditions, including polymyositis, Sjögren syndrome, or vasculitis. This review presents an overview of the etiology of and biomarkers associated with sarcoidosis. We then provide a detailed description of the rheumatologic manifestations of sarcoidosis and present a treatment algorithm based on current clinical evidence for patients with sarcoid arthritis. The discussion will focus on characteristic findings in patients with sarcoid arthritis, osseous involvement in sarcoidosis, and sarcoid myopathy. Arthritic conditions that sometimes coexist with sarcoidosis are described as well. We present two cases of sarcoidosis with rheumatologic manifestations. Our intent is to encourage a multidisciplinary, translational approach to meet the challenges and difficulties in understanding and treating sarcoidosis.

  13. Rheumatologic Manifestations of Sarcoidosis

    PubMed Central

    Sweiss, Nadera J.; Patterson, Karen; Sawaqed, Ray; Jabbar, Umair; Korsten, Peter; Hogarth, Kyle; Wollman, Robert; Garcia, Joe G.N.; Niewold, Timothy B.; Baughman, Robert P.

    2012-01-01

    Sarcoidosis is a systemic, clinically heterogeneous disease characterized by the development of granulomas. Any organ system can be involved, and patients may present with any number of rheumatologic symptoms. There are no U.S. Food and Drug Administration–approved therapies for the treatment of sarcoidosis. Diagnosing sarcoidosis becomes challenging, particularly when its complications cause patients’ symptoms to mimic other conditions, including polymyositis, Sjögren syndrome, or vasculitis. This review presents an overview of the etiology of and biomarkers associated with sarcoidosis. We then provide a detailed description of the rheumatologic manifestations of sarcoidosis and present a treatment algorithm based on current clinical evidence for patients with sarcoid arthritis. The discussion will focus on characteristic findings in patients with sarcoid arthritis, osseous involvement in sarcoidosis, and sarcoid myopathy. Arthritic conditions that sometimes coexist with sarcoidosis are described as well. We present two cases of sarcoidosis with rheumatologic manifestations. Our intent is to encourage a multidisciplinary, translational approach to meet the challenges and difficulties in understanding and treating sarcoidosis. PMID:20665396

  14. Eosinophilic granuloma: MRI manifestations.

    PubMed

    Beltran, J; Aparisi, F; Bonmati, L M; Rosenberg, Z S; Present, D; Steiner, G C

    1993-01-01

    The appearance on magnetic resonance imaging (MRI) of 16 cases of pathologically proven eosinophilic granuloma were reviewed retrospectively and correlated with the radiographic appearance of the lesion. The most common MR appearance (ten cases) was a focal lesion, surrounded by an extensive, ill-defined bone marrow and soft tissue reaction with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, considered to represent bone marrow and soft tissue edema (the flare phenomenon). The MRI manifestations of eosinophilic granuloma, especially during the early stages, are nonspecific, and may stimulate an aggressive lesion such as osteomyelitis or Ewings sarcoma, or other benign bone tumors such as osteoid osteoma or chondroblastoma. PMID:8480200

  15. Ophthalmic manifestations postlightning strike.

    PubMed

    Dhillon, Permesh Singh; Gupta, Mohit

    2015-01-01

    Various ophthalmic complications affecting the anterior and posterior segments have been identified due to lightning strike. We report the first case of an indirect lightning-induced full thickness macular hole formation in the UK as evidenced by slit lamp examination and optical coherence tomography (OCT) scan in a 77-year-old woman presenting with sudden visual loss in her right eye and thermal skin injury affecting her scalp. Her best corrected visual acuities were LogMAR 0.46 and 0.12 in the right and left eyes, respectively. There were no other ocular manifestations observed in either eye. She was initially managed conservatively with non-steroidal anti-inflammatory drug eye drops but surgery was later advised due to minimal changes in the visual acuity and macular hole on follow-up. OCT scanning is important in diagnosing macular holes, which usually warrant surgical intervention. PMID:25827914

  16. [Skin manifestations of monoclonal gammopathies].

    PubMed

    Hello, M; Barbarot, S; Néel, A; Connault, J; Graveleau, J; Durant, C; Decaux, O; Hamidou, M

    2014-01-01

    Whatever their aetiology, monoclonal gammopathies can be associated to several clinical features. Mechanisms are various and sometimes unknown. Skin is frequently involved and may represent a challenging diagnosis. Indeed, skin manifestations are either the presenting features and isolated, or at the background of a systemic syndrome. Our objective was to review the various skin manifestations that have been associated with monoclonal gammopathies.

  17. Hyaline fibromatosis syndrome: cutaneous manifestations*

    PubMed Central

    Marques, Silvio Alencar; Stolf, Hamilton Ometto; Polizel, Juliana Ocanha; Munhoz, Tânia; Brandão, Marcela Calixto; Marques, Mariangela Esther Alencar

    2016-01-01

    Hyaline fibromatosis syndrome is the current name for clinical manifestations of diseases previously known as “infantile systemic hyalinosis” and “juvenile hyaline fibromatosis”. The authors report representative clinical cases of each one of the above subtypes with emphasis on cutaneous manifestations and difficulties for early diagnosis in this syndrome, essentially of multidisciplinary approach. PMID:27192526

  18. Quantum manifestations of Nekhoroshev stability

    NASA Astrophysics Data System (ADS)

    Fontanari, Daniele; Fassò, Francesco; Sadovskií, Dmitrií A.

    2016-09-01

    We uncover quantum manifestations of classical Nekhoroshev theory of resonant dynamics using a simple quantum system of two coupled angular momenta with conserved equal magnitudes which corresponds to a perturbed classical integrable anisochronous Hamiltonian system.

  19. Oral Manifestations of Secondary Syphilis.

    PubMed

    de Paulo, Luiz Fernando Barbosa; Servato, João Paulo Silva; Oliveira, Maiolino Thomaz Fonseca; Durighetto, Antonio Francisco; Zanetta-Barbosa, Darceny

    2015-06-01

    Known as "the great imitator," secondary syphilis may clinically manifest itself in myriad ways, involving different organs including the oral mucosa, and mimicking, both clinically and histologically, several diseases, thereby making diagnosis a challenge for clinicians. We highlight the clinical aspects of oral manifestation in 7 patients with secondary syphilis. Clinicians should consider secondary syphilis in the differential diagnosis of ulcerative and/or white oral lesions.

  20. Oral Manifestations of Secondary Syphilis.

    PubMed

    de Paulo, Luiz Fernando Barbosa; Servato, João Paulo Silva; Oliveira, Maiolino Thomaz Fonseca; Durighetto, Antonio Francisco; Zanetta-Barbosa, Darceny

    2015-06-01

    Known as "the great imitator," secondary syphilis may clinically manifest itself in myriad ways, involving different organs including the oral mucosa, and mimicking, both clinically and histologically, several diseases, thereby making diagnosis a challenge for clinicians. We highlight the clinical aspects of oral manifestation in 7 patients with secondary syphilis. Clinicians should consider secondary syphilis in the differential diagnosis of ulcerative and/or white oral lesions. PMID:25892249

  1. Neurological manifestations of filarial infections.

    PubMed

    Bhalla, Devender; Dumas, Michel; Preux, Pierre-Marie

    2013-01-01

    Filarial infections cause a huge public health burden wherever they are endemic. These filaria may locate anywhere in the human body. Their manifestations and pathogenic mechanisms, except the most common ones, are rarely investigated systematically. Their neurological manifestations, however, are being increasingly recognized particularly with onchocerciasis or Loa loa infections, Wuchereria bancrofti, or Mansonella perstans. The risk of developing these manifestations may also increase in cases that harbor multiple filariasis or coinfections, for instance as with Plasmodium. The microfilaria of Onchocerca and Loa loa are seen in cerebrospinal fluid. The pathogenesis of neurological manifestations of these infections is complex; however, pathogenic reactions may be caused by mechanical disruption, e.g., degeneration often followed by granulomas, causing fibrosis or mass effects on other tissues, vascular lesions, e.g., vascular block of cerebral vessels, or disordered inflammatory responses resulting in meningitis, encephalitis or localized inflammatory responses. The chances of having neurological manifestations may also depend upon the frequency and"heaviness"of infection over a lifetime. Hence, this type of infection should no longer be considered a disease of the commonly affected areas but one that may produce systemic effects or other manifestations, and these should be considered in populations where they are endemic. PMID:23829914

  2. Neurocutaneous Manifestations of Genetic Mosaicism

    PubMed Central

    van Steensel, Maurice A. M.

    2015-01-01

    Genetic mosaicism is defined as the presence of two or more genetically distinct cell populations in a single individual. Ever more disorders are found to be manifestations of mosaicism and together constitute a significant proportion of the morbidity confronting pediatric specialists. An emerging category is that of overgrowth syndromes with skin manifestations and neurological or developmental abnormalities, such as the well-known Proteus syndrome. In recent years, we have seen dramatic advances in our understanding of these disorders and we now know the genetic basis of many of them. This has profound consequences for diagnosis, counselling, and even treatment, with therapies targeted to specific pathways becoming available for clinical use. Recognizing such overgrowth syndromes, therefore, is more important than ever. Fortunately, their skin manifestations can provide important diagnostic clues when evaluated in the entire phenotypic context. In this review, I provide an overview of the most frequently seen mosaic neurocutaneous phenotypes and discuss their molecular basis. PMID:27617125

  3. Neurocutaneous Manifestations of Genetic Mosaicism.

    PubMed

    van Steensel, Maurice A M

    2015-09-01

    Genetic mosaicism is defined as the presence of two or more genetically distinct cell populations in a single individual. Ever more disorders are found to be manifestations of mosaicism and together constitute a significant proportion of the morbidity confronting pediatric specialists. An emerging category is that of overgrowth syndromes with skin manifestations and neurological or developmental abnormalities, such as the well-known Proteus syndrome. In recent years, we have seen dramatic advances in our understanding of these disorders and we now know the genetic basis of many of them. This has profound consequences for diagnosis, counselling, and even treatment, with therapies targeted to specific pathways becoming available for clinical use. Recognizing such overgrowth syndromes, therefore, is more important than ever. Fortunately, their skin manifestations can provide important diagnostic clues when evaluated in the entire phenotypic context. In this review, I provide an overview of the most frequently seen mosaic neurocutaneous phenotypes and discuss their molecular basis. PMID:27617125

  4. Psychiatric manifestations in cerebrotendinous xanthomatosis

    PubMed Central

    Fraidakis, M J

    2013-01-01

    Cerebrotendinous xanthomatosis (CTX) is a rare and severe, but treatable, inborn disorder of bile acid biosynthesis and sterol storage with autosomal recessive inheritance and variable clinical presentation. CTX treatment consists of chenodeoxycholic acid and must be started as early as possible to prevent permanent disability. Psychiatric manifestations are rare and non-specific, and often lead to significant diagnostic and treatment delay. Therefore, better recognition of the gamut of psychiatric manifestations in CTX can diminish the risk of misdiagnosis and irreversible neurological deterioration. We hereby describe the psychiatric features in CTX. A complete review of all published cases of CTX in the medical literature was undertaken and the case reports with psychiatric presentation were collected and analyzed. We also describe the psychiatric features in relation to the neurological semeiology in six patients with CTX diagnosed at the La Salpêtrière Hospital. We conclude that psychiatric manifestations in CTX follow a bimodal/bitemporal pattern, appearing early in the disease course in the form of a behavioral/personality disorder associated with learning difficulties or mental retardation, or manifesting in advanced disease in the setting of dementia as rich neuropsychiatric syndromes, such as frontal, orbitofrontal or frontotemporal syndromes of cortico-subcortical dementia encompassing behavioral/personality disturbance, affective/mood disorders or psychotic disorders. Behavioral/personality disturbance in childhood or adolescence, especially when accompanied by learning difficulties, should therefore lead to further investigation to exclude CTX, as early diagnosis and treatment is critical for prognosis. PMID:24002088

  5. [Acromegaly and its orthopedic manifestations].

    PubMed

    Helal, B

    1983-08-01

    A review of the orthopaedic manifestations of acromegaly has been carried out from a study of literature. A further special study of 83 acromegalic patients reveals that about one third consulted the specialist for surgical intervention. In 14 of 28 patients a surgical intervention became necessary. The most frequent cases in question were nerve decompression. Recovery and success rate were always satisfactory. PMID:6415942

  6. 40 CFR 262.21 - Manifest tracking numbers, manifest printing, and obtaining manifests.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... instructions in the appendix to 40 CFR part 262 must appear legibly on the back of the copies of the manifest... AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The...): “Designated facility to destination State (if required)”. (ii) Page 2: “Designated facility to generator...

  7. Porphyria and its neurologic manifestations.

    PubMed

    Tracy, Jennifer A; Dyck, P James B

    2014-01-01

    Porphyrias are rare disorders resulting from a defect in the heme biosynthetic pathway. They can produce significant disease of both the peripheral and central nervous systems, in addition to other organ systems, with acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria as the subtypes associated with neurologic manifestations. The presence of a motor-predominant peripheral neuropathy (axonal predominant), accompanied by gastrointestinal distress and neuropsychiatric manifestations, should be a strong clue to the diagnosis of porphyria. Clinical confirmation can be made through evaluation of urine porphyrins during an exacerbation of disease. While hematin is helpful for acute treatment, long-term effective management requires avoidance of overstimulation of the cytochrome P450 pathway, as well as other risk factor control.

  8. [Ophthalmological manifestations of cerebrovascular disease].

    PubMed

    Gallego Culleré, J; Herrera, M; Navarro, Mc

    2008-01-01

    Transient or persistent loss of vision in one eye is a common and distinctive manifestation of occlusive vascular disease. Occasionally, both eyes are involved together or sequentially, with temporary or even permanent blindness. The internal carotid arteries supply blood to the organ of vision; therefore pathologies of those arteries caused by arteriosclerosis may have a direct influence on its functioning. The most common syndromes are temporary (amaurosis fugax) or constant reduction of visual acuity. In fundus examination central retinal artery occlusion and branch retinal artery occlusion are the most common diagnosis, while retinal vein occlusion, anterior ischemic optic neuropathy, ocular ischemic syndrome are less common. There are many clinical ophtlamological manifestations due to vascular brain damage. Proper recognition and diagnosis of the disease may protect the patient against serious life-threatening complications such as stroke. PMID:19169299

  9. Orofacial manifestations of systemic sclerosis.

    PubMed

    Veale, B J; Jablonski, R Y; Frech, T M; Pauling, J D

    2016-09-23

    Systemic sclerosis (SSc) is a multisystem disease of unknown aetiology characterised by microangiopathy, dysregulated immune function and tissue remodelling, which commonly involves the oral cavity. Orofacial manifestations of SSc contribute greatly to overall disease burden and yet are regularly overlooked and under-treated. This may reflect a pre-occupation amongst rheumatology clinicians on potentially life-threatening internal organ involvement, but is also a consequence of insufficient engagement between rheumatologists and dental professionals. A high proportion of SSc patients report difficulty accessing a dentist with knowledge of the disease and there is recognition amongst dentists that this could impact negatively on patient care. This review shall describe the clinical features and burden of orofacial manifestations of SSc and the management of such problems. The case is made for greater collaborative working between rheumatologists and dental professionals with an interest in SSc in both the research and clinical setting. PMID:27659631

  10. Clinical Manifestations of Portal Hypertension

    PubMed Central

    Al-Busafi, Said A.; McNabb-Baltar, Julia; Farag, Amanda; Hilzenrat, Nir

    2012-01-01

    The portal hypertension is responsible for many of the manifestations of liver cirrhosis. Some of these complications are the direct consequences of portal hypertension, such as gastrointestinal bleeding from ruptured gastroesophageal varices and from portal hypertensive gastropathy and colopathy, ascites and hepatorenal syndrome, and hypersplenism. In other complications, portal hypertension plays a key role, although it is not the only pathophysiological factor in their development. These include spontaneous bacterial peritonitis, hepatic encephalopathy, cirrhotic cardiomyopathy, hepatopulmonary syndrome, and portopulmonary hypertension. PMID:23024865

  11. Systemic diseases with cutaneous manifestations.

    PubMed

    Merchant, S R; Taboada, J

    1995-07-01

    The purpose of this article is to briefly discuss the following cutaneous manifestations of selected systemic diseases: poxvirus; feline leukemia virus (FeLV); feline immunodeficiency virus (FIV); herpesvirus; calcivirus; pseudorabies; plague; tularemia; toxoplasmosis; leishmania; hypothyroidism; hyperthyroidism; hyperadrenocorticism; diabetes mellitus; acromegaly; thallium poisoning; pancreatic disease; hypereosinophilic syndrome; mucopolysaccharidosis; and pansteatitis. Recognition of these cutaneous signs may help alert the clinician to the possibility of an internal disorder so that the appropriate diagnostic tests can be considered. PMID:8525575

  12. Rheumatic manifestations of diabetes mellitus.

    PubMed

    Lebiedz-Odrobina, Dorota; Kay, Jonathan

    2010-11-01

    DM is associated with various musculoskeletal manifestations. The strength of this relationship varies among the various musculoskeletal disorders; the associations are based mostly on epidemiologic data. For most of these conditions, definitive pathophysiologic correlates are lacking.Hand and shoulder disorders occur more frequently than other musculoskeletal manifestations of DM. Recognition of the association between DM and shoulder adhesive capsulitis, DD, and stenosing flexor tenosynovitis facilitates their correct diagnosis in the setting of DM and prompt initiation of appropriate treatment, which may include optimizing glycemic control. Conversely, awareness and identification of the characteristic musculoskeletal manifestations of DM may facilitate earlier diagnosis of DM and initiation of glucose-lowering therapy to retard the development of diabetic complications.Much less has been published about the musculoskeletal complications of DM than about its micro- and macrovascular complications. Prospective case-control cohort studies are needed to establish the true prevalence of musculoskeletal complications of DM and the metabolic syndrome, especially in this era of tighter glycemic control.The potential relationship between DM and the development of OA needs to be clarified in large, prospective, case-control cohort studies. The effect on musculoskeletal manifestations of various therapeutic regimens to manage DM should be studied prospectively. Treatment regimens for some musculoskeletal conditions associated with DM, such as DISH, should be studied in larger prospective, randomized,controlled clinical trials.At the molecular level, further studies are warranted to clarify the potential contribution of AGEs and adipokines to the development of OA and diabetic musculoskeletal syndromes, such as shoulder adhesive capsulitis, DD, stenosing flexor tenosynovitis, and LJM. Identification of such molecular targets for therapy would promote the development of

  13. Hepatic Manifestations in Hematological Disorders

    PubMed Central

    Murakami, Jun

    2013-01-01

    Liver involvement is often observed in several hematological disorders, resulting in abnormal liver function tests, abnormalities in liver imaging studies, or clinical symptoms presenting with hepatic manifestations. In hemolytic anemia, jaundice and hepatosplenomegaly are often seen mimicking liver diseases. In hematologic malignancies, malignant cells often infiltrate the liver and may demonstrate abnormal liver function test results accompanied by hepatosplenomegaly or formation of multiple nodules in the liver and/or spleen. These cases may further evolve into fulminant hepatic failure. PMID:23606974

  14. Imaging Manifestations of Thoracic Tuberculosis.

    PubMed

    Restrepo, Carlos Santiago; Katre, Rashmi; Mumbower, Amy

    2016-05-01

    Despite significant improvements in the diagnosis and treatment of tuberculosis achieved during the last 3 decades, tuberculosis still remains one of the deadliest communicable diseases worldwide. Tuberculosis is still present in all regions of the world, with a more significant impact in developing countries. This article reviews the most common imaging manifestations of primary and postprimary tuberculosis, their complications, and the critical role of imaging in the diagnosis and follow-up of affected patients.

  15. Atypical manifestations of dengue fever.

    PubMed

    Pawaria, Arti; Mishra, Devendra; Juneja, Monica; Meena, Jagdish

    2014-06-01

    We reviewed case records of 40 in-patients (22 boys) with serologically confirmed dengue fever between 1st October and 30th November, 2013. Severe dengue was seen in 30, out of which 12 (30%) had compensated shock. Splenomegaly (6,15%) and encephalopathy (4,10%) were the commonest atypical features. Atypical manifestations of dengue fever were more common than that reported in the past.

  16. Parasitic Diseases With Cutaneous Manifestations.

    PubMed

    Ash, Mark M; Phillips, Charles M

    2016-01-01

    Parasitic diseases result in a significant global health burden. While often thought to be isolated to returning travelers, parasitic diseases can also be acquired locally in the United States. Therefore, clinicians must be aware of the cutaneous manifestations of parasitic diseases to allow for prompt recognition, effective management, and subsequent mitigation of complications. This commentary also reviews pharmacologic treatment options for several common diseases. PMID:27621348

  17. [Neuropsychiatric manifestations ushering pernicious anemia].

    PubMed

    Mrabet, S; Ellouze, F; Ellini, S; Mrad, M F

    2015-12-01

    Biermer disease or pernicious anemia is an autoimmune atrophic gastritis characterized by the lack of secretion of gastric intrinsic factor. This leads to an insufficient absorption of vitamin B12 in the ileum. Clinical manifestations are mainly hematologic. Neuropsychiatric manifestations are known but are less frequent especially early in the disease. Inaugural neuropsychiatric arrays are rare and various thus making diagnosis difficult. In this article, we report through two clinical cases different neuropsychiatric manifestations revealing pernicious anemia. Mrs. C.O., aged 56, presented after surgery for gallstones, an acute psychiatric array associated with gait disorders. She had no history of neurological or psychiatric problems. The psychiatric interview revealed delirious syndrome, depressive symptoms and anxiety. Neurological examination noted a flaccid paraplegia with peripheral neuropathic syndrome and myoclonus in the upper limbs. At the full blood count, a macrocytosis (VGM: 112.2fl) without anemia was found. The level of vitamin B12 in the blood was low. Cerebro-spinal MRI was suggestive of a neuro-Biermer and showed hyper signal in the cervical cord on T2-weighted sagittal section. In axial section, hyper signal appears at the posterior columns in the form of V. There were no brain abnormalities. A sensorimotor axonal polyneuropathy was diagnosed. The patient received vitamin B12 intramuscularly for ten days associated with neuroleptic treatment. Mrs. R.M., aged 40, was brought to the psychiatry consultation for acute behavioral disorders progressively worsening over a month. An anxiety syndrome, depressive syndrome and delirious syndrome were identified. Neurological examination showed a posterior cordonal syndrome with quadripyramidal syndrome. Full blood count showed a macrocytic anemia. Serum B12 level was collapsed. Cerebro-spinal MRI was normal. She received vitamin B12 with clinical and biological improvement. Features of pernicious anemia

  18. [Neuropsychiatric manifestations ushering pernicious anemia].

    PubMed

    Mrabet, S; Ellouze, F; Ellini, S; Mrad, M F

    2015-12-01

    Biermer disease or pernicious anemia is an autoimmune atrophic gastritis characterized by the lack of secretion of gastric intrinsic factor. This leads to an insufficient absorption of vitamin B12 in the ileum. Clinical manifestations are mainly hematologic. Neuropsychiatric manifestations are known but are less frequent especially early in the disease. Inaugural neuropsychiatric arrays are rare and various thus making diagnosis difficult. In this article, we report through two clinical cases different neuropsychiatric manifestations revealing pernicious anemia. Mrs. C.O., aged 56, presented after surgery for gallstones, an acute psychiatric array associated with gait disorders. She had no history of neurological or psychiatric problems. The psychiatric interview revealed delirious syndrome, depressive symptoms and anxiety. Neurological examination noted a flaccid paraplegia with peripheral neuropathic syndrome and myoclonus in the upper limbs. At the full blood count, a macrocytosis (VGM: 112.2fl) without anemia was found. The level of vitamin B12 in the blood was low. Cerebro-spinal MRI was suggestive of a neuro-Biermer and showed hyper signal in the cervical cord on T2-weighted sagittal section. In axial section, hyper signal appears at the posterior columns in the form of V. There were no brain abnormalities. A sensorimotor axonal polyneuropathy was diagnosed. The patient received vitamin B12 intramuscularly for ten days associated with neuroleptic treatment. Mrs. R.M., aged 40, was brought to the psychiatry consultation for acute behavioral disorders progressively worsening over a month. An anxiety syndrome, depressive syndrome and delirious syndrome were identified. Neurological examination showed a posterior cordonal syndrome with quadripyramidal syndrome. Full blood count showed a macrocytic anemia. Serum B12 level was collapsed. Cerebro-spinal MRI was normal. She received vitamin B12 with clinical and biological improvement. Features of pernicious anemia

  19. Clinical manifestations of sleep apnea

    PubMed Central

    2015-01-01

    Obstructive sleep apnea (OSA) may manifest in a number of ways from subtle intrusion into daily life to profound sleepiness, snoring, witnessed apneas and other classic symptoms. Although there is increasing evidence suggesting OSA can adversely affect health in a variety of ways, this disorder remains underdiagnosed. The most well-escribed health consequences of OSA relate to the cardiovascular system. Hypertension and arrhythmias have a strong association with OSA, and evidence suggests that treatment of OSA in patients with refractory hypertension and in patients planning cardioversion for atrial fibrillation may be of particularly importance. Significant associations between heart failure and OSA as well as complex sleep apnea have also been well-described. Cerebrovascular insult, impaired neurocognition, and poorly controlled mood disorder are also associated with in OSA. Therapy for OSA may ameliorate atherosclerotic progression and improve outcomes post-cerebrovascular accident (CVA). OSA should be considered in patients complaining of poor concentration at work, actual or near-miss motor vehicle accidents, and patients with severe sleepiness as a component of their co-morbid mood disorders. The metabolic impact of OSA has also been studied, particularly in relation to glucose homeostasis. Also of interest is the potential impact OSA has on lipid metabolism. The adverse effect untreated OSA has on glucose tolerance and lipid levels has led to the suggestion that OSA is yet another constituent of the metabolic syndrome. Some of these metabolic derangements may be related to the adverse effects untreated OSA has on hepatic health. The cardiovascular, neurocognitive, and metabolic manifestations of OSA can have a significant impact on patient health and quality of life. In many instances, evidence exists that therapy not only improves outcomes in general, but also modifies the severity of co-morbid disease. To mitigate the long-term sequela of this disease

  20. Clinical manifestations of food allergy.

    PubMed

    Perry, Tamara T; Pesek, Robbie D

    2013-06-01

    Adverse reactions to foods are a diverse group of clinical syndromes resulting from immunologic and non-immunologic responses to food ingestion. Symptoms can range from mild, self-limiting reactions to severe, life-threatening reactions depending on the mechanism. This review primarily focuses on the clinical manifestations of immunologically derived adverse food reactions or food allergies.The true prevalence of food allergy is unknown. Up to 25% of the general population believes that they may be allergic to some food; however, the actual prevalence of food allergy diagnosed by a provider appears to be 1.5% to 2% of the adult population and approximately 6% to 8% of children. This discrepancy makes it imperative that clinicians are aware of the different food allergy syndromes. With a clear understanding of the clinical manifestations of food allergies, an accurate diagnosis and treatment plan can be formulated. Failing to do so may result in unnecessary dietary restrictions that may adversely affect nutritional status, growth, and quality of life.Most food allergic reactions are secondary to a limited number of foods, and the most common foods causing allergic reactions in children include milk, egg, peanuts, tree nuts, and fish. In adolescents and adults, allergies to peanuts, tree nuts, fish, and shellfish are most prevalent. Food allergies can result from immunoglobulin E (IgE)-mediated, non-IGE-mediated, or mixed IgE/non-IgE mechanisms. The purpose of this review is to discuss the clinical manifestations of each of these types of food allergy.

  1. Unusual manifestations of premenstrual syndrome.

    PubMed

    Hsiao, Mei-Chun; Liu, Chai-Yih

    2007-02-01

    The authors have identified six unusual manifestations of premenstrual syndrome: (i) typical premenstrual dysphoric disorder symptoms during ovulation; (ii) upper airway infection in the premenstrual phase that remits after onset of menstruation; (iii) recurrent perimenstrual erythematous and edematous skin plaques; (iv) hypomanic or manic state 2-3 days before onset of menstruation; (v) auditory hallucinations and delusion of reference, present only premenstrually; and (vi) remitted bipolar disorder presenting with premenstrual psychotic features. Psychiatrists should be aware of menstrual cycle-related changes in mental illness, physical illness, or both in women of reproductive age, if cyclic trends are suspected.

  2. Musculoskeletal manifestations of endocrine disorders.

    PubMed

    Boswell, Stephanie B; Patel, Dakshesh B; White, Eric A; Gottsegen, Christopher J; Forrester, Deborah M; Masih, Sulabha; Matcuk, George R

    2014-01-01

    Endocrine disorders can lead to disturbances in numerous systems within the body, including the musculoskeletal system. Radiological evaluation of these conditions can demonstrate typical appearances of the bones and soft tissues. Knowledge of these patterns can allow the radiologist to suggest a diagnosis that may not be clinically apparent. This review will highlight the typical musculoskeletal findings of acromegaly, hypercortisolism, hyperthyroidism, hypothyroidism, hyperparathyroidism, pseudo- and pseudopseudohypoparathyroidism, and diabetes mellitus. The radiological manifestations of each of these endocrine disorders, along with a brief discussion of the pathophysiology and clinical implications, will be discussed. PMID:24642251

  3. Cutaneous manifestations of chikungunya fever.

    PubMed

    Seetharam, K A; Sridevi, K; Vidyasagar, P

    2012-01-01

    Chikungunya fever, a re-emerging RNA viral infection produces different cutaneous manifestations in children compared to adults. 52 children with chikungunya fever, confirmed by positive IgM antibody test were seen during 2009-2010. Pigmentary lesions were common (27/52) followed by vesiculobullous lesions (16/52) and maculopapular lesions (14/52). Vesiculobullous lesions were most common in infants, although rarely reported in adults. Psoriasis was exacerbated in 4 children resulting in more severe forms. In 2 children, guttate psoriasis was observed for the first time.

  4. Rheumatic manifestations in diabetic patients

    PubMed Central

    Serban, AL; Udrea, GF

    2012-01-01

    Diabetes mellitus (DM), a worldwide high prevalence disease, is associated with a large variety of rheumatic manifestations. For most of these affections, pathophysiologic correlations are not well established. Some of them, such as diabetic cheiroarthropathy, neuropathic arthritis, diabetic amyotrophy, diabetic muscle infraction, are considered intrinsic complications of DM. For others, like diffuse idiopathic skeletal hyperostosis or reflex sympathetic dystrophy, DM is considered a predisposing condition. In most cases, these affections cause pain and disability, affecting the quality of life of diabetic patients, but once correctly diagnosed, they often respond to the treatment, that generally requires a multidisciplinary team. This article reviews some epidemiological, clinical, diagnostic and therapeutic aspects of these conditions. PMID:23049626

  5. Cutaneous manifestations of breast cancer.

    PubMed

    Tan, Antoinette R

    2016-06-01

    Breast cancer may present with cutaneous symptoms. The skin manifestations of breast cancer are varied. Some of the more common clinical presentations of metastatic cutaneous lesions from breast cancer will be described. Paraneoplastic cutaneous dermatoses have been reported as markers of breast malignancy and include erythema gyratum repens, acquired ichthyosis, dermatomyositis, multicentric reticulohistiocytosis, and hypertrichosis lanuginosa acquisita. Mammary Paget's disease, often associated with an underlying breast cancer, and Cowden syndrome, which has an increased risk of breast malignancy, each have specific dermatologic findings. Recognition of these distinct cutaneous signs is important in the investigation of either newly diagnosed or recurrent breast cancer. PMID:27178684

  6. Atypical Cutaneous Manifestations in Syphilis.

    PubMed

    Ivars Lleó, M; Clavo Escribano, P; Menéndez Prieto, B

    2016-05-01

    Although the diversity of the clinical manifestations of syphilis is well-known, atypical presentations can also occur. Such atypical presentations are associated with a high risk of transmission as a result of diagnostic confusion and treatment delays owing to the disease's ability to mimic other common skin diseases, deviate from classic clinical presentations, and adopt unique forms. Cases of atypical syphilis have been described most frequently in patients with concomitant human immunodeficiency virus (HIV) infection. Because the incidence of syphilis has been growing over recent years -particularly in patients with HIV co-infection- dermatologists need to be familiar with the less well-known clinical presentations of this venereal disease.

  7. Cutaneous manifestation of gastrointestinal disease

    PubMed Central

    Kerstetter, Justin

    2016-01-01

    The gastrointestinal (GI) and cutaneous systems are closely linked in origin. Skin manifestations are frequently seen as a part of different GI syndromes. Gastroenterologists play an important role in recognizing the symptoms, patient workup and arriving at appropriate diagnoses, often in consultation with dermatologists. This review discusses the diseases with both cutaneous and intestinal involvement. Hereditary polyposis GI cancers, hereditary nonpolyposis colorectal cancers (CRCs), hamartomatous disorders, and inflammatory bowel disease (IBD) are reviewed with emphasis on the genetic basis, diagnostic, histologic findings, screening modalities, and therapeutic options. PMID:27034812

  8. Coronal manifestations of preflare activity

    NASA Technical Reports Server (NTRS)

    Schmahl, E. J.; Webb, D. F.; Woodgate, B.; Waggett, P.; Bentley, R.; Hurford, G.; Schadee, A.; Schrijver, J.; Harrison, R.; Martens, P.

    1986-01-01

    A variety of coronal manifestations of precursors or preheating for flares are discussed. Researchers found that almost everyone with a telescope sees something before flares. Whether an all-encompassing scenario will ever be developed is not at all clear at present. The clearest example of preflare activity appears to be activated filaments and their manifestations, which presumably are signatures of a changing magnetic field. But researchers have seen two similar eruptions, one without any evidence of emerging flux (Kundu et al., 1985) and the other with colliding poles (Simon et al., 1984). While the reconnection of flux is generally agreed to be required to energize a flare, the emergence of flux from below (at least on short timescales and in compact regions) does not appear to be a necessary condition. In some cases the cancelling of magnetic flux (Martin, 1984) by horizontal motions instead may provide the trigger (Priest, 1985) Researchers found similarities and some differences between these and previous observations. The similarities, besides the frequent involvement of filaments, include compact, multiple precursors which can occur both at and near (not at) the flare site, and the association between coronal sources and activity lower in the atmosphere (i.e., transition zone and chromosphere).

  9. 40 CFR 761.210 - Manifest discrepancies.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... Section 761.210 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.210 Manifest discrepancies. (a) Manifest discrepancies are differences between the quantity or type of PCB waste designated on the manifest or...

  10. 40 CFR 761.210 - Manifest discrepancies.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... Section 761.210 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.210 Manifest discrepancies. (a) Manifest discrepancies are differences between the quantity or type of PCB waste designated on the manifest or...

  11. 40 CFR 761.210 - Manifest discrepancies.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... Section 761.210 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.210 Manifest discrepancies. (a) Manifest discrepancies are differences between the quantity or type of PCB waste designated on the manifest or...

  12. 40 CFR 761.215 - Manifest discrepancies.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... Section 761.215 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.215 Manifest discrepancies. (a) Manifest... quantity or type of PCB waste designated on the manifest or shipping paper, and the quantity and type...

  13. Hematologic manifestations of celiac disease

    PubMed Central

    Halfdanarson, Thorvardur R.; Litzow, Mark R.; Murray, Joseph A.

    2007-01-01

    Celiac disease is a common systemic disorder that can have multiple hematologic manifestations. Patients with celiac disease may present to hematologists for evaluation of various hematologic problems prior to receiving a diagnosis of celiac disease. Anemia secondary to malabsorption of iron, folic acid, and/or vitamin B12 is a common complication of celiac disease and many patients have anemia at the time of diagnosis. Celiac disease may also be associated with thrombocytosis, thrombocytopenia, leukopenia, venous thromboembolism, hyposplenism, and IgA deficiency. Patients with celiac disease are at increased risk of being diagnosed with lymphoma, especially of the T-cell type. The risk is highest for enteropathy-type T-cell lymphoma (ETL) and B-cell lymphoma of the gut, but extraintestinal lymphomas can also be seen. ETL is an aggressive disease with poor prognosis, but strict adherence to a gluten-free diet may prevent its occurrence. PMID:16973955

  14. Neurologic manifestations of lyme disease.

    PubMed

    Halperin, John J

    2011-08-01

    Borrelia burgdorferi infection, the tick-borne spirochetosis known as Lyme disease or Lyme borreliosis, involves the nervous system (neuroborreliosis) in 10% to 15% of patients. Common manifestations include lymphocytic meningitis, cranial neuritis, mononeuropathy multiplex, and painful radiculoneuritis. Rare patients develop inflammation in the brain or spinal cord. Regardless of the form of involvement, neuroborreliosis can be microbiologically cured in virtually all patients using standard 2- to 4-week antimicrobial regimens. Oral regimens appear to be as effective as parenteral ones in most instances. Although patients ill with Lyme disease may have concomitant cognitive or memory difficulty, these symptoms are not specific to neuroborreliosis and, when present in isolation, should not be viewed as suggestive of this diagnosis. When present as part of Lyme disease, they do not require additional or different treatment.

  15. Atypical Cutaneous Manifestations in Syphilis.

    PubMed

    Ivars Lleó, M; Clavo Escribano, P; Menéndez Prieto, B

    2016-05-01

    Although the diversity of the clinical manifestations of syphilis is well-known, atypical presentations can also occur. Such atypical presentations are associated with a high risk of transmission as a result of diagnostic confusion and treatment delays owing to the disease's ability to mimic other common skin diseases, deviate from classic clinical presentations, and adopt unique forms. Cases of atypical syphilis have been described most frequently in patients with concomitant human immunodeficiency virus (HIV) infection. Because the incidence of syphilis has been growing over recent years -particularly in patients with HIV co-infection- dermatologists need to be familiar with the less well-known clinical presentations of this venereal disease. PMID:26708562

  16. [Pulmonary manifestations in rheumatoid arthritis].

    PubMed

    Morawska, Justyna; Domysławska, Izabela; Bagrowska, Magdalena; Sierakowski, Stanislaw

    2015-01-01

    Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by destructive cartilages, bones and other structures formed joints. RA belongs to connective tissue diseases represented by systemic nature, internal illness, extra-articular features and rapidly progress of atherosceirosis. The extra-articular complications cause the reduction of patient longevity. The frequency of symptoms in patient with RA and respiratory disorders occur in 10-20% of cases. Pulmonary complications are the second most common cause of premature of patient deaths. Respiratory disorders associated with RA are devided into 3 groups: infection, lung disease caused by drugs and pulmonary manifestation connected by RA. These last affect interstitial tissue, bronchioli, pulmonary vessels, pleura, also are presented by pulmonary rheumatoid nodules and pulmonary hypertension.

  17. Oral manifestations in transplant patients

    PubMed Central

    Nappalli, Deepika; Lingappa, Ashok

    2015-01-01

    Organ transplantation is a widely undertaken procedure and has become an important alternative for the treatment of different end-stage organ diseases that previously had a poor prognosis. The field of organ transplant and hematopoietic stem cell transplant is developing rapidly. The increase in the number of transplant recipients also has an impact on oral and dental services. Most of the oral problems develop as a direct consequence of drug-induced immunosuppression or the procedure itself. These patients may present with oral complaints due to infections or mucosal lesions. Such lesions should be identified, diagnosed, and treated. New treatment strategies permit continuous adaptation of oral care regimens to the changing scope of oral complications. The aim of this review is to analyze those oral manifestations and to discuss the related literature. PMID:26005458

  18. Cutaneous manifestations of lung cancer.

    PubMed

    Owen, Cindy England

    2016-06-01

    Skin findings can serve as a clue to internal disease. In this article, cutaneous manifestations of underlying lung malignancy are reviewed. Paraneoplastic dermatoses are rare, but when recognized early, can lead to early diagnosis of an underlying neoplasm. Malignancy-associated dermatoses comprise a broad group of hyperproliferative and inflammatory disorders, disorders caused by tumor production of hormonal or metabolic factors, autoimmune connective tissue diseases, among others. In this review, paraneoplastic syndromes associated with lung malignancy are discussed, including ectopic ACTH syndrome, bronchial carcinoid variant syndrome, secondary hypertrophic osteoarthropathy/digital clubbing, erythema gyratum repens, malignant acanthosis nigricans, sign of Leser-Trélat, tripe palms, hypertrichosis lanuginosa, acrokeratosis paraneoplastica, and dermatomyositis. PMID:27178690

  19. [Parathyroid dysfunction and rheumatic manifestations].

    PubMed

    Frey, D P

    2011-11-01

    Parathyroid dysfunction, leading to severe clinical symptoms and radiographic changes, has decreased over the last years due to routine laboratory checks including serum calcium levels. Thus, abnormal calcium levels are detected early in the course of the disease and the underlying cause treated accordingly. Hyperparathyroidism often leads to osteoporosis and low-trauma fractures. When evaluating secondary osteoporosis analysis of calcium, phosphate and intact parathyroid hormone levels are mandatory. Osteitis fibrosa cystica and brown tumors are less frequent findings of hyperparathyroidism. However, in patients with arthritis or bone symptoms, hyperparathyroidism has to be evaluated as a possible reason. Other manifestations of hyperparathyroidism include myopathy, tendon ruptures and unspecific symptoms of the muscles and skeleton. Gout as well as pseudogout may be associated with hyperparathyroidism. Hypoparathyroidism may cause musculoskeletal diseases mimicking ankylosing spondylitis or diffuse idiopathic skeletal hyperostosis. Myopathies are sometimes induced by hypoparathyroidism. An association between systemic lupus erythematosus and hypoparathyroidism seems to exist.

  20. Gastrointestinal manifestations of food allergies.

    PubMed

    Wolfe, Jaime Liou; Aceves, Seema S

    2011-04-01

    The rates of eosinophilic gastrointestinal disorders appear to be increasing. The most common of these is eosinophilic esophagitis (EoE) which is a clinicopathologic condition consisting of characteristic symptoms and endoscopic features accompanied by a pan-esophageal, acid resistant epithelial eosinophilia of greater than equal to 15 per high power field. Typical symptoms include dysphagia and abdominal pain. Typical endoscopic features include pallor, plaques, furrows, concentric rings. Complications include food impactions and strictures. EoE resolution with food elimination diets provides evidence that EoE is a food-antigen driven process. In vitro and microarray studies have identified specific immunologic factors underlying EoE pathogenesis. Other gastrointestinal manifestations of food intolerances/allergy include food protein induced enterocolitis syndrome.

  1. Caudal Cingulate Infarction Manifesting Astasia

    PubMed Central

    Satow, Takeshi; Komuro, Taro; Kobayashi, Akira

    2014-01-01

    Introduction Astasia is a rare presenting symptom of stroke, usually known as ‘thalamic astasia’, induced by a lesion in the ventrolateral thalamus. We report a case of caudal cingulate infarction manifesting astasia. Case Presentation A 58-year-old male presented with inability to sit, stand and walk (astasia). No apparent motor weakness was noticed in the extremities. MRI revealed cerebral infarction in the caudal cingulate gyrus, which was located between the vertical commissure anterior (VCA) line and vertical commissure posterior (VPC) line. His symptoms persisted for 1 year to a lesser degree. Conclusion Lesions in the caudal cingulate gyrus can present with astasia. The responsible lesion is located in the cingulate gyrus between the VCA and VPC line, which might correspond to the caudal cingulate zone in humans. We should keep in mind that astasia can be a presenting symptom of stroke. PMID:24575027

  2. Psoriasis: pathophysiology and oral manifestations.

    PubMed

    Zhu, J F; Kaminski, M J; Pulitzer, D R; Hu, J; Thomas, H F

    1996-06-01

    Psoriasis is a chronic, remitting and relapsing inflammatory skin disorder with a strong genetic predisposition. Psoriasis affects 1-3% of the world's population in their early lives representing a disabling condition with significant social and economic impact. Despite a great deal of research on the etiology and tissue destruction mechanisms, the disease is not well understood. The purpose of this paper is to provide current information from the literature with a special focus on oral manifestations. The major signs and symptoms presented in the oral environment of a psoriasis patient may include geographic tongue, fissure tongue, gingival and/or mucosal lesions. Inflammatory temporomandibular joint lesions have been reported in less than 5% of psoriasis patients. Multiple treatment strategies, be they topical or systemic, have been applied to these patients for symptom relief but not for cure.

  3. 78 FR 11877 - Announcement of Requirements Gathering Meetings for the Electronic Manifest (e-Manifest) System

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-02-20

    ... agency begins the planning stage of the e-Manifest system development process. EPA envisions that e... electronically completing and transmitting manifests through a national, centralized e-Manifest system (71 FR... planning phase to begin the development and ultimately the deployment of the e-Manifest system by 2015....

  4. Clinical Manifestations of Spontaneous Pneumomediastinum

    PubMed Central

    Park, Soo Jin; Park, Ji Ye; Jung, Joonho; Park, Seong Yong

    2016-01-01

    Background Spontaneous pneumomediastinum (SPM) is an uncommon disorder with only a few reported clinical studies. The goals of this study were to investigate the clinical manifestations and the natural course of SPM, as well as examine the current available treatment options for SPM. Methods We retrospectively reviewed 91 patients diagnosed with SPM between January 2008 and June 2015. Results The mean age of the patients was 22.7±13.2 years, and 67 (73.6%) were male. Chest pain (58, 37.2%) was the predominant symptom. The most frequent precipitating factor before developing SPM was a cough (15.4%), but the majority of patients (51, 56.0%) had no precipitating factors. Chest X-ray was diagnostic in 44 patients (48.4%), and chest computed tomography (CT) showed mediastinal air in all cases. Esophagography (10, 11.0%), esophagoduodenoscopy (1, 1.1%), and bronchoscopy (5, 5.5%) were performed selectively due to clinical suspicion, but no abnormal findings that implicated organ injury were documented. Twelve patients (13.2%) were discharged after a visit to the emergency room, and the others were admitted and received conservative treatment. The mean length of hospital stay was 3.0±1.6 days. There were no complications related to SPM except for recurrence in 2 patients (2.2%). Conclusion SPM responds well to conservative treatment and follows a benign natural course. Hospitalization and aggressive treatment can be performed in selective cases. PMID:27525238

  5. Ocular manifestations of frontonasal dysplasia.

    PubMed

    Roarty, J D; Pron, G E; Siegel-Bartelt, J; Posnick, J C; Buncic, J R

    1994-01-01

    The ophthalmologic findings associated with frontonasal dysplasia have not been defined previously in a large series of untreated children. We reviewed the ophthalmic manifestations of a series of patients with frontonasal dysplasia who were seen as part of their craniofacial evaluation. All had undergone a complete ophthalmologic examination before any manipulation of either the orbits or the soft tissues of the orbital contents. From 1986 to 1991, 23 patients with frontonasal dysplasia were seen; ophthalmologic abnormalities were found in 20 (87 percent). Abnormalities included significant refractive errors, strabismus, nystagmus, and eyelid ptosis. Three patients had amblyopia, a treatable cause of visual loss, from strabismus or anisometropia. Ten eyes in seven patients (30 percent) had severe structural anomalies, such as optic nerve hypoplasia, optic nerve colobomas, microphthalmia, cataract, corneal dermoid, or inflammatory retinopathy, that resulted in an acuity of 20/100 or worse. The high incidence of ocular abnormalities indicates that early assessment by an ophthalmologist should be part of the initial evaluation of patients with frontonasal dysplasia to detect treatable visual or ocular problems. PMID:8278482

  6. Cutaneous manifestations of genitourinary malignancy.

    PubMed

    Raghavan, Derek

    2016-06-01

    Genitourinary cancers are associated with a range of cutaneous syndromes, which can reflect direct metastatic spread, non-metastatic manifestations of malignancy or the consequences of treatment. More than 220,000 new cases of prostate cancer occur each year in the United States, and thus the associations with cutaneous involvement are quite well documented-rare metastatic spread, vasculitic and hemorrhagic syndromes. Cancers of the bladder and kidney may be associated with direct cutaneous metastases, vasculitic syndromes, hereditary leiomyomatosis, and other familial syndromes. Testicular cancer occasionally metastasizes to the skin but more commonly is associated with the dysplastic nevus (multiple atypical nevus) syndrome. A structured approach to history-taking, examination, and investigation is essential for optimal management, especially when these syndromes precede the diagnosis of a known malignancy. A brief review of the more common iatrogenic cutaneous complications is provided, and includes Raynaud's phenomenon, purpura, rash, hand-foot syndrome, the consequences of marrow failure, and bleomycin-induced pigmentation. PMID:27178687

  7. Esophageal manifestations of celiac disease.

    PubMed

    Lucendo, A J

    2011-09-01

    Celiac disease (CD) may often be associated with various motor disorders affecting the different segments of the digestive tract, including the esophagus. Although it has not been universally reported, some available evidences indicate that pediatric and adult celiac patients could manifest a higher frequency of esophagitis and gastroesophageal reflux disease-related symptoms compared to nonceliac patients. In addition, several published studies have consistently shown the efficacy of a gluten-free diet in rapidly controlling esophageal symptoms and in preventing their recurrence. Since the participation of gluten in the esophageal symptoms of CD seems clear, its intimate mechanisms have yet to be elucidated, and several hypothesis have been proposed, including the specific immune alterations characterizing CD, the reduction in nutrient absorption determining the arrival of intact gluten to distal gastrointestinal segments, and various dysregulations in the function of gastrointestinal hormones and peptides. Recent studies have suggested the existence of a possible relationship between CD and eosinophilic esophagitis, which should be more deeply investigated.

  8. Rheumatic manifestations of pituitary tumors.

    PubMed

    Stavrou, S; Kleinberg, D L

    2001-10-01

    Pituitary tumors may cause rheumatologic problems as a result of under production or overproduction of one pituitary hormone. Excessive growth hormone causes destruction of cartilage by a direct action. Facial and acral changes and arthralgias may be some of the first symptoms of acromegaly. The arthritis associated with acromegaly is often devastating. Carpal tunnel syndrome is very common in patients with acromegaly. Adrenocorticotropin (ACTH) has indirect effects via the action of glucocorticoid on bones, muscles, and the immune system. Proximal muscle weakness is a characteristic feature of Cushing's syndrome. Patients with Cushing's syndrome commonly have osteopenia and osteoporosis that lead to an increase in bone fractures. Avascular necrosis is associated with exogenous steroid administration. The effects of too much glucocorticoid or too rapid withdrawal can be severe. Gonadotropins act via the gonadal steroids and protect bone mass from loss. Prolactin is less involved in rheumatologic disease; the data for which are limited in humans. Pituitary tumors can have manifestations similar to rheumatologic disorders and should be included in the differential diagnosis of these diseases.

  9. Skeletal manifestations of infantile scurvy.

    PubMed

    Brickley, Megan; Ives, Rachel

    2006-02-01

    Recent investigations of human skeletal material from the historic St. Martin's cemetery, England, found a range of abnormal lesions in six infants that are almost certainly related to scurvy. Porous and proliferative bone lesions affecting the cranial bones and scapulae were found, and this paper presents images obtained using both macroscopic and scanning electron microscope examination of the lesions. Previous work on infantile scurvy (Ortner et al., 1997-2001) relied heavily on changes at the sphenoid, which is often missing in archaeological bone, so the identification of changes attributable to scurvy on other cranial bones and the scapulae is encouraging. The ability to recognize changes related to scurvy on a range of bones will ensure an enhanced potential for recognition of this disease in future research involving archaeological bone. Research on historical documents from Birmingham dating to the eighteenth and nineteenth centuries, combined with the probable cases of scurvy identified, supports the view that the paucity of cases of infantile scurvy from the archaeological record reflects a lack of understanding and recognition of bone manifestations, rather than a lack of occurrence in this period. Changes linked to scurvy were only found in infants from the poorer sections of the community from St. Martin's, and this is almost certainly linked to patterns of food consumption and may be related to shortages of potatoes, due to blight, experienced during this period.

  10. Extrahepatic manifestations of hepatitis E virus.

    PubMed

    Kamar, Nassim; Marion, Olivier; Abravanel, Florence; Izopet, Jacques; Dalton, Harry R

    2016-04-01

    Hepatitis E virus can cause acute, fulminant and chronic hepatitis and has been associated with a range of extrahepatic manifestations. Guillain-Barré syndrome, neuralgic amyotrophy and encephalitis are the main neurological manifestations associated with acute and chronic hepatitis E virus infection. Renal injuries have been also reported, including membranoproliferative glomerulonephritis with or without cryoglobulinemia and membranous glomerulonephritis. Acute pancreatitis, haematological disorders and other autoimmune extrahepatic manifestations of hepatitis E virus, such as myocarditis and thyroiditis, have been also reported. In this comprehensive article, we review all published reports describing hepatitis E virus-associated extrahepatic manifestations.

  11. Extrahepatic manifestations of hepatitis E virus.

    PubMed

    Kamar, Nassim; Marion, Olivier; Abravanel, Florence; Izopet, Jacques; Dalton, Harry R

    2016-04-01

    Hepatitis E virus can cause acute, fulminant and chronic hepatitis and has been associated with a range of extrahepatic manifestations. Guillain-Barré syndrome, neuralgic amyotrophy and encephalitis are the main neurological manifestations associated with acute and chronic hepatitis E virus infection. Renal injuries have been also reported, including membranoproliferative glomerulonephritis with or without cryoglobulinemia and membranous glomerulonephritis. Acute pancreatitis, haematological disorders and other autoimmune extrahepatic manifestations of hepatitis E virus, such as myocarditis and thyroiditis, have been also reported. In this comprehensive article, we review all published reports describing hepatitis E virus-associated extrahepatic manifestations. PMID:27005692

  12. Ocular manifestations of HIV infection.

    PubMed Central

    Jabs, D A

    1995-01-01

    all stages of HIV infection. Fifty-six percent of the cases of ocular toxoplasmosis had simultaneous toxoplasmic cerebritis. Ocular toxoplasmosis responded to standard anti-microbial therapy. Varicella zoster virus retinitis, when manifested by the acute retinal necrosis (ARN) syndrome, responded to intravenous acyclovir therapy. Conversely, in a limited number of patients with the progressive outer retinal necrosis syndrome, the disease responded poorly to intravenous acyclovir therapy, but appeared to respond to combination foscarnet and acyclovir therapy. Neuro-ophthalmic lesions were present in 6% of the patients with AIDS. The most common cause of a neuro-ophthalmic lesion was cryptococcal meningitis, and 25% of the patients with cryptococcal meningitis developed a neuro-ophthalmic complication. CONCLUSIONS: Ocular manifestations are common in patients with AIDS. CMV retinitis represented a major vision-threatening problem in these patients. While available therapy was successful in initially controlling the retinitis, the phenomenon of relapse resulted in some degree of long-term visual loss. Preservation of the patient's visual acuity in at least one eye was generally successful. Other opportunistic ocular infections were substantially less common than CMV retinitis but require aggressive therapy. Images FIGURE 1 FIGURE 2 FIGURE 3 FIGURE 4 FIGURE 5 FIGURE 6 PMID:8719695

  13. [Hemolytic uremic syndrome. Clinical manifestations. Treatment].

    PubMed

    Exeni, Ramón A

    2006-01-01

    Clinical manifestation are described in children with epidemic HUS. The intestinal involvement in the prodromic period, is outlined and the most common disturbances such acute renal failure, thrombocytopenia, hemolytic anemia, leucocitosis hypertension, neurological, pancreatic and cardiac manifestations are described. We discuss the acid-base and electrolyte disturbances, metabolic acidosis, hyponatremia, hyperkalemia. The etiopathogenic treatment and the control of renal sequelae are also discussed.

  14. Teaching about Manifest Destiny: Clarifying the Concept.

    ERIC Educational Resources Information Center

    Chiodo, John J.

    2000-01-01

    Provides information on Manifest Destiny and the expansionist ideals of the United States, especially during the 19th century. Offers a lesson plan that will help students understand the concept of Manifest Destiny, including an assessment activity, how to expand the lesson, an appendix, and references. (CMK)

  15. Unusual clinical manifestations of dengue virus infection.

    PubMed

    George, R; Liam, C K; Chua, C T; Lam, S K; Pang, T; Geethan, R; Foo, L S

    1988-12-01

    Four recent cases of dengue fever with severe, unusual clinical manifestations are described. Two of these cases had features of fulminant hepatitis and encephalopathy; one of these cases was fatal. The two remaining cases showed hepatitis with renal impairment. The significance and importance of these unusual manifestations of dengue disease are discussed.

  16. Clinical manifestations of zinc deficiency.

    PubMed

    Prasad, A S

    1985-01-01

    The essentiality of zinc for humans was recognized in the early 1960s. The causes of zinc deficiency include malnutrition, alcoholism, malabsorption, extensive burns, chronic debilitating disorders, chronic renal diseases, following uses of certain drugs such as penicillamine for Wilson's disease and diuretics in some cases, and genetic disorders such as acrodermatitis enteropathica and sickle cell disease. In pregnancy and during periods of growth the requirement of zinc is increased. The clinical manifestations in severe cases of zinc deficiency include bullous-pustular dermatitis, alopecia, diarrhea, emotional disorder, weight loss, intercurrent infections, hypogonadism in males; it is fatal if unrecognized and untreated. A moderate deficiency of zinc is characterized by growth retardation and delayed puberty in adolescents, hypogonadism in males, rough skin, poor appetite, mental lethargy, delayed wound healing, taste abnormalities, and abnormal dark adaptation. In mild cases of zinc deficiency in human subjects, we have observed oligospermia, slight weight loss, and hyperammonemia. Zinc is a growth factor. Its deficiency adversely affects growth in many animal species and humans. Inasmuch as zinc is needed for protein and DNA synthesis and for cell division, it is believed that the growth effect of zinc is related to its effect on protein synthesis. Whether or not zinc is required for the metabolism of somatomedin needs to be investigated in the future. Testicular functions are affected adversely as a result of zinc deficiency in both humans and experimental animals. This effect of zinc is at the end organ level; the hypothalamic-pituitary axis is intact in zinc-deficient subjects. Inasmuch as zinc is intimately involved in cell division, its deficiency may adversely affect testicular size and thus affect its functions. Zinc is required for the functions of several enzymes and whether or not it has an enzymatic role in steroidogenesis is not known at present

  17. [Lyme disease--clinical manifestations and treatment].

    PubMed

    Stock, Ingo

    2016-05-01

    Lyme disease (Lyme borreliosis) is a systemic infectious disease that can present in a variety of clinical manifestations. The disease is caused by a group of spirochaetes--Borrelia burgdorferi sensu lato or Lyme borrelia--that are transmitted to humans by the bite of Ixodes ticks. Lyme disease is the most common arthropode-borne infectious disease in many European countries including Germany. Early localized infection is typically manifested by an erythema migrans skin lesion, in rarer cases as a borrelial lymphocytoma. The most common early disseminated manifestation is (early) neuroborreliosis. In adults, neuroborreliosis appears typically as meningoradiculoneuritis. Neuroborreliosis in children, however, is typically manifested by meningitis. In addition, multiple erythema migrans lesions and Lyme carditis occur relatively frequently. The most common manifestation oflate Lyme disease is Lyme arthritis. Early manifestations (and usually also late manifestations) of Lyme disease can be treated successfully by application of suitable antibacterial agents. For the treatment of Lyme disease, doxycycline, certain penicillins such as amoxicillin and some cephalosporins (ceftriaxone, cefotaxime, cefuroxime axetil) are recommended in current guidelines. A major challenge is the treatment of chronic, non-specific disorders, i. e., posttreatment Lyme disease syndrome and "chronic Lyme disease". Prevention of Lyme disease is mainly accomplished by protecting against tick bites. Prophylactic administration of doxycycline after tick bites is generally not recommended in Germany. There is no vaccine available for human beings.

  18. [Lyme disease--clinical manifestations and treatment].

    PubMed

    Stock, Ingo

    2016-05-01

    Lyme disease (Lyme borreliosis) is a systemic infectious disease that can present in a variety of clinical manifestations. The disease is caused by a group of spirochaetes--Borrelia burgdorferi sensu lato or Lyme borrelia--that are transmitted to humans by the bite of Ixodes ticks. Lyme disease is the most common arthropode-borne infectious disease in many European countries including Germany. Early localized infection is typically manifested by an erythema migrans skin lesion, in rarer cases as a borrelial lymphocytoma. The most common early disseminated manifestation is (early) neuroborreliosis. In adults, neuroborreliosis appears typically as meningoradiculoneuritis. Neuroborreliosis in children, however, is typically manifested by meningitis. In addition, multiple erythema migrans lesions and Lyme carditis occur relatively frequently. The most common manifestation oflate Lyme disease is Lyme arthritis. Early manifestations (and usually also late manifestations) of Lyme disease can be treated successfully by application of suitable antibacterial agents. For the treatment of Lyme disease, doxycycline, certain penicillins such as amoxicillin and some cephalosporins (ceftriaxone, cefotaxime, cefuroxime axetil) are recommended in current guidelines. A major challenge is the treatment of chronic, non-specific disorders, i. e., posttreatment Lyme disease syndrome and "chronic Lyme disease". Prevention of Lyme disease is mainly accomplished by protecting against tick bites. Prophylactic administration of doxycycline after tick bites is generally not recommended in Germany. There is no vaccine available for human beings. PMID:27348896

  19. Paraneoplastic cutaneous manifestations: concepts and updates*

    PubMed Central

    da Silva, Josenilson Antônio; Mesquita, Kleyton de Carvalho; Igreja, Ana Carolina de Souza Machado; Lucas, Isabella Cristina Rodrigues Naves; Freitas, Aline Ferreira; de Oliveira, Sandra Maximiano; Costa, Izelda Maria Carvalho; Campbell, Iphis Tenfuss

    2013-01-01

    The skin often signals systemic changes. Some neoplastic diseases that affect internal organs may trigger several cutaneous manifestations. Although these dermatoses are relatively unusual, the recognition of some typical paraneoplastic dermatoses may lead to the early diagnosis of a neoplasm and determine a better prognosis. In this review article, we discuss the paraneoplastic cutaneous manifestations strongly associated with neoplasms, which include acanthosis nigricans maligna, tripe palms, erythema gyratum repens, Bazex syndrome, acquired hypertrichosis lanuginosa, necrolytic migratory erythema, Leser-Trélat sign and paraneoplastic pemphigus. We also review the clinical manifestations of each condition and include updated knowledge on disease pathogenesis. PMID:23538999

  20. Genetic alterations in syndromes with oral manifestations.

    PubMed

    Anuthama, Krishnamurthy; Prasad, Harikrishnan; Ramani, Pratibha; Premkumar, Priya; Natesan, Anuja; Sherlin, Herald J

    2013-11-01

    Ever since Gregor Johan Mendel proposed the law of inheritance, genetics has transcended the field of health and has entered all walks of life in its application. Thus, the gene is the pivoting factor for all happenings revolving around it. Knowledge of gene mapping in various diseases would be a valuable tool in prenatally diagnosing the condition and averting the future disability and stigma for the posterity. This article includes an array of genetically determined conditions in patients seen at our college out-patient department with complete manifestation, partial manifestation and array of manifestations not fitting into a particular syndrome.

  1. Genetic alterations in syndromes with oral manifestations

    PubMed Central

    Anuthama, Krishnamurthy; Prasad, Harikrishnan; Ramani, Pratibha; Premkumar, Priya; Natesan, Anuja; Sherlin, Herald J.

    2013-01-01

    Ever since Gregor Johan Mendel proposed the law of inheritance, genetics has transcended the field of health and has entered all walks of life in its application. Thus, the gene is the pivoting factor for all happenings revolving around it. Knowledge of gene mapping in various diseases would be a valuable tool in prenatally diagnosing the condition and averting the future disability and stigma for the posterity. This article includes an array of genetically determined conditions in patients seen at our college out-patient department with complete manifestation, partial manifestation and array of manifestations not fitting into a particular syndrome. PMID:24379857

  2. Mucocutaneous manifestations of helminth infections: Nematodes.

    PubMed

    Lupi, Omar; Downing, Christopher; Lee, Michael; Pino, Livia; Bravo, Francisco; Giglio, Patricia; Sethi, Aisha; Klaus, Sidney; Sangueza, Omar P; Fuller, Claire; Mendoza, Natalia; Ladizinski, Barry; Woc-Colburn, Laila; Tyring, Stephen K

    2015-12-01

    In the 21st century, despite increased globalization through international travel for business, medical volunteerism, pleasure, and immigration/refugees into the United States, there is little published in the dermatology literature regarding the cutaneous manifestations of helminth infections. Approximately 17% of travelers seek medical care because of cutaneous disorders, many related to infectious etiologies. This review will focus on the cutaneous manifestations of helminth infections and is divided into 2 parts: part I focuses on nematode infections, and part II focuses on trematode and cestode infections. This review highlights the clinical manifestations, transmission, diagnosis, and treatment of helminth infections. Nematodes are roundworms that cause diseases with cutaneous manifestations, such as cutaneous larval migrans, onchocerciasis, filariasis, gnathostomiasis, loiasis, dracunculiasis, strongyloidiasis, ascariasis, streptocerciasis, dirofilariasis, and trichinosis. Tremadotes, also known as flukes, cause schistosomiasis, paragonimiasis, and fascioliasis. Cestodes (tapeworms) are flat, hermaphroditic parasites that cause diseases such as sparganosis, cysticercosis, and echinococcus.

  3. Important cutaneous manifestations of inflammatory bowel disease

    PubMed Central

    Trost, L; McDonnell, J

    2005-01-01

    Inflammatory bowel disease (IBD) has many extraintestinal manifestations. Cutaneous manifestations are usually related to the activity of the bowel disease but may have an independent course. Anyone presenting with IBD should be examined for cutaneous manifestations. Pyoderma gangrenosum is a severe painful ulcerating disease that requires moist wound management and, in the absence of secondary infection, systemic corticosteroids, cyclosporine, or both. Infliximab may also be used. Erythema nodosum is a common cause of tender red nodules of the shins. Management includes leg elevation, NSAIDs, and potassium iodide. Oral manifestations of IBD include aphthous stomatitis, mucosal nodularity (cobblestoning), and pyostomatitis vegetans. Treatment should be directed both at the cutaneous lesions and at the underlying systemic condition. PMID:16143688

  4. Musculoskeletal manifestations of the antiphospholipid syndrome.

    PubMed

    Noureldine, M H A; Khamashta, M A; Merashli, M; Sabbouh, T; Hughes, G R V; Uthman, I

    2016-04-01

    The scope of clinical and laboratory manifestations of the antiphospholipid syndrome (APS) has increased dramatically since its discovery in 1983, where any organ system can be involved. Musculoskeletal complications are consistently reported in APS patients, not only causing morbidity and mortality, but also affecting their quality of life. We reviewed all English papers on APS involvement in the musculoskeletal system using Google Scholar and Pubmed; all reports are summarized in a table in this review. The spectrum of manifestations includes arthralgia/arthritis, avascular necrosis of bone, bone marrow necrosis, complex regional pain syndrome type-1, muscle infarction, non-traumatic fractures, and osteoporosis. Some of these manifestations were reported in good quality studies, some of which showed an association between aPL-positivity and the occurrence of these manifestations, while others were merely described in case reports. PMID:26923284

  5. 40 CFR 761.208 - Obtaining manifests.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... print the manifest under 40 CFR 262.21 (c) and (e). A registered source may be a: (i) State agency; (ii) Commercial printer; (iii) PCB waste generator, transporter or, designated facility; or (iv) PCB waste...

  6. 40 CFR 761.208 - Obtaining manifests.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... print the manifest under 40 CFR 262.21 (c) and (e). A registered source may be a: (i) State agency; (ii) Commercial printer; (iii) PCB waste generator, transporter or, designated facility; or (iv) PCB waste...

  7. Current oral manifestations of HIV infection.

    PubMed

    Navazesh, M

    2001-02-01

    The oral manifestations of human immunodeficiency virus infection have changed drastically since the introduction of the highly active anti-retroviral therapy (HAART) in developed countries. Recent studies have documented significant reductions in morbidity and mortality rates among HIV-infected patients on HAART. This article focuses on the latest information about the oral manifestations of HIV infection and will discuss the impact of HAART.

  8. Pulmonary manifestations of inflammatory bowel disease

    PubMed Central

    Ji, Xiao-Qing; Wang, Li-Xia; Lu, De-Gan

    2014-01-01

    Extraintestinal manifestations of inflammatory bowel disease (IBD) are a systemic illness that may affect up to half of all patients. Among the extraintestinal manifestations of IBD, those involving the lungs are relatively rare and often overlooked. However, there is a wide array of such manifestations, spanning from airway disease to lung parenchymal disease, thromboembolic disease, pleural disease, enteric-pulmonary fistulas, pulmonary function test abnormalities, and adverse drug reactions. The spectrum of IBD manifestations in the chest is broad, and the manifestations may mimic other diseases. Although infrequent, physicians dealing with IBD must be aware of these conditions, which are sometimes life-threatening, to avoid further health impairment of the patients and to alleviate their symptoms by prompt recognition and treatment. Knowledge of these manifestations in conjunction with pertinent clinical data is essential for establishing the correct diagnosis and treatment. The treatment of IBD-related respiratory disorders depends on the specific pattern of involvement, and in most patients, steroids are required in the initial management. Corticosteroids, both systemic and aerosolized, are the mainstay therapeutic approach, while antibiotics must also be administered in the case of infectious and suppurative processes, whose sequelae sometimes require surgical intervention. PMID:25309080

  9. Extra-articular Manifestations in Rheumatoid Arthritis

    PubMed Central

    Cojocaru, Manole; Cojocaru, Inimioara Mihaela; Silosi, Isabela; Vrabie, Camelia Doina; Tanasescu, R

    2010-01-01

    ABSTRACT Rheumatoid arthritis (RA) is a systemic autoimmune disease whose main characteristic is persistent joint inflammation that results in joint damage and loss of function. Although RA is more common in females, extra-articular manifestations of the disease are more common in males. The extra-articular manifestations of RA can occur at any age after onset. It is characterised by destructive polyarthritis and extra-articular organ involvement, including the skin, eye, heart, lung, renal, nervous and gastrointestinal systems. The frequence of extra-articular manifestations in RA differs from one country to another. Extra-articular organ involvement in RA is more frequently seen in patients with severe, active disease and is associated with increased mortality. Incidence and frequence figures for extra-articular RA vary according to study design. Extra-articular involvement is more likely in those who have RF and/or are HLA-DR4 positive. Occasionally, there are also systemic manifestations such as vasculitis, visceral nodules, Sjögren's syndrome, or pulmonary fibrosis present. Nodules are the most common extra-articular feature, and are present in up to 30%; many of the other classic features occur in 1% or less in normal clinic settings. Sjögren's syndrome, anaemia of chronic disease and pulmonary manifestations are relatively common – in 6-10%, are frequently present in early disease and are all related to worse outcomes measures of rheumatoid disease in particular functional impairment and mortality. The occurrence of these systemic manifestations is a major predictor of mortality in patients with RA. This paper focuses on extra-articular manifestations, defined as diseases and symptoms not directly related to the locomotor system. PMID:21977172

  10. 77 FR 54818 - Polychlorinated Biphenyls (PCBs): Revisions to Manifesting Regulations

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-09-06

    ... affected entities Electric Power Distribution.... 221122 Generators of PCB waste. Transportation and... requirements, which uses the Resource Conservation and Recovery Act (RCRA) Uniform Hazardous Waste Manifest... manifesting requirements for PCBs under TSCA to the manifesting requirements for hazardous waste under...

  11. 14 CFR 121.693 - Load manifest: All certificate holders.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 14 Aeronautics and Space 3 2010-01-01 2010-01-01 false Load manifest: All certificate holders. 121... manifest: All certificate holders. The load manifest must contain the following information concerning the... certificate holder....

  12. Nocturnal manifestations of atypical parkinsonian disorders.

    PubMed

    Bhidayasiri, Roongroj; Jitkritsadakul, Onanong; Colosimo, Carlo

    2014-01-01

    Although nocturnal disturbances are increasingly recognized as an integral part of the continuum of daytime manifestations of Parkinson's disease (PD), there is still little evidence in the medical literature to support the occurrence of these complex phenomena in patients with atypical parkinsonian disorders (APDs). Based on the anatomical substrates in APDs, which are considered to be more extensive outside the basal ganglia than in PD, we might expect that patients with APDs encounter the whole range of nocturnal disturbances, including motor, sleep disorders, autonomic dysfunctions, and neuropsychiatric manifestations at a similar, or even greater, frequency than in PD. This article is a review of the current literature on the problems at nighttime of patients with progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration, and dementia with Lewy bodies. MEDLINE, life science journals and online books were searched by querying appropriate key words. Reports were included if the studies were related to nocturnal manifestations in APDs. Forty articles fulfilled the selection criteria. Differences between these symptoms in APDs and PD are highlighted, given the evidence available about each manifestation. This analysis of nocturnal manifestations of APDs suggests the need for future studies to address these issues to improve the quality of life not only of patients with APDs but the caregivers who encounter the challenges of supporting these patients on a daily basis.

  13. Pulmonary manifestations of renal cell carcinoma.

    PubMed

    Agrawal, Abhinav; Sahni, Sonu; Iftikhar, Asma; Talwar, Arunabh

    2015-12-01

    Renal cell carcinoma (RCC) accounts for majority of all primary renal neoplasms. Classic manifestations of RCC include the triad of flank pain, hematuria and a palpable renal mass. Patients with RCC can develop various extra renal manifestations including involvements of the lungs, inferior vena cava, liver and the bones. The pulmonary manifestations of renal cell carcinoma include metastatic disease including endobronchial, pleural, parenchymal or lymph node metastasis, pleural effusion or hemothorax. Pulmonary embolism and tumor embolism is another common manifestation of renal cell carcinoma. RCC is a highly vascular tumor and can cause pulmonary arterio-venous fistulas leading to high output failure. Rarely, RCC can also present with paraneoplastic presentations including cough or bilateral diaphragm paralysis. Drugs used to treat RCC have been associated with drug related pneumonitis and form an important differential diagnosis in patients with RCC on therapy presenting with shortness of breath. In this review we discuss the various pulmonary manifestations of RCC. A high index of suspicion with these presentations can lead to an early diagnosis and assist in instituting an appropriate intervention. PMID:26525375

  14. Novel Cutaneous Manifestations of Pleuroparenchymal Fibroelastosis.

    PubMed

    Lowther, Christopher M; Morrison, Annie O; Candelario, Nicole M; Khalafbeigi, Sheva; Cockerell, Clay J

    2016-10-01

    Pleuroparenchymal fibroelastosis (PPFE) is a rare progressive disease that manifests as parenchymal fibrosis of the upper lobe and pleura. Approximately 100 cases have been reported. Cutaneous manifestations of PPFE have not previously been described. Diagnosis is dependent on histologic identification of fibrosis with atypical elastic fibers, necessitating an invasive peripheral lung wedge biopsy.A 68-year-old male with a history of pleuroparenchymal fibroelastosis presented with an asymptomatic, telangiectatic erythematous eruption on bilateral lower extremities. Biopsies demonstrated a subtle perivascular infiltrate with marked increase in atypical elastic fibers, similar to the elastosis in the patient's lungs.This is the first documented case of cutaneous manifestations in PPFE. Clinicians need to be aware that cutaneous eruptions clinically simulating telangiectasia macularis eruptiva perstans but lacking a mast cell infiltrate histologically, may have increased abnormal elastic fibers. Thus, early recognition of these lesions in patients with an undefined restrictive lung disorder, may facilitate the diagnosis of PPFE in some patients.

  15. Novel Cutaneous Manifestations of Pleuroparenchymal Fibroelastosis.

    PubMed

    Lowther, Christopher M; Morrison, Annie O; Candelario, Nicole M; Khalafbeigi, Sheva; Cockerell, Clay J

    2016-10-01

    Pleuroparenchymal fibroelastosis (PPFE) is a rare progressive disease that manifests as parenchymal fibrosis of the upper lobe and pleura. Approximately 100 cases have been reported. Cutaneous manifestations of PPFE have not previously been described. Diagnosis is dependent on histologic identification of fibrosis with atypical elastic fibers, necessitating an invasive peripheral lung wedge biopsy.A 68-year-old male with a history of pleuroparenchymal fibroelastosis presented with an asymptomatic, telangiectatic erythematous eruption on bilateral lower extremities. Biopsies demonstrated a subtle perivascular infiltrate with marked increase in atypical elastic fibers, similar to the elastosis in the patient's lungs.This is the first documented case of cutaneous manifestations in PPFE. Clinicians need to be aware that cutaneous eruptions clinically simulating telangiectasia macularis eruptiva perstans but lacking a mast cell infiltrate histologically, may have increased abnormal elastic fibers. Thus, early recognition of these lesions in patients with an undefined restrictive lung disorder, may facilitate the diagnosis of PPFE in some patients. PMID:27643829

  16. [Neurological manifestations of giant cell arteritis].

    PubMed

    Grachev, Yu V

    2016-01-01

    The article describes clinical, including neurological manifestations, of giant cell arteritis (GCA) - granulomatous vasculitis of large and medium-sized vessels, predominantly craniofacial, including precerebral and cerebral, arteries. Histopathological features of GCA are illustrated by the schemes of panarteritis and «postarteritis» (proliferative and fibrotic changes in the intima, underlying the development of cerebrovascular disorders). The main clinical manifestations of GCA are described as 3 groups of symptoms: general constitutional symptoms; manifestations of vasculitis of craniofacial, precerebral and cerebral arteries; polymyalgia rheumaticа. The authors present their own version of the taxonomy of visual disturbances in patients with GCA. Diagnostic steps in patients with suggestive signs of GCA are described. Therapeutic regimens of use of glucocorticoids for suggestion/diagnosis of GCA are presented.

  17. Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

    PubMed Central

    Nakamura, Yutaro; Suda, Takafumi

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF. PMID:27625576

  18. Lupus as a paraneoplastic manifestation of cholangiocarcinoma.

    PubMed

    González Amores, Yolanda; Hernando Rebollar, Sofía; Casado Bernabeu, Aida

    2016-05-01

    Tumors originating in the digestive system, like those in other areas, whether solid or otherwise, may present with extradigestive manifestations in the setting of a paraneoplastic syndrome. Systemic lupus erythematosus (SLE) is an autoimmune condition most commonly involving women of childbearing age. On occasion it represents a paraneoplastic manifestation heralding a primary tumor. Cancer suspicion is therefore a key element for newly diagnosed SLE cases with nonstandard epidemiology even in the absence of suggestive symptoms, and digestive tumors should be included in the differential diagnosis. PMID:26925842

  19. Ocular manifestations of feline viral diseases.

    PubMed

    Stiles, Jean

    2014-08-01

    Feline viral diseases are common and cats can be presented with a variety of clinical manifestations. Ocular disease associated with viral pathogens is not unusual, particularly with viruses causing upper respiratory tract disease in cats, such as feline herpesvirus type 1 and feline calicivirus. These agents mainly cause ocular surface disease. Other viruses, such as feline immunodeficiency virus and feline coronavirus, can cause uveitis, while feline leukemia virus can induce ocular lymphosarcoma. This review covers the most common viral pathogens of cats that cause ocular manifestations, the specific features of the ocular diseases caused by these viruses and therapeutic recommendations.

  20. Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

    PubMed Central

    Nakamura, Yutaro; Suda, Takafumi

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF.

  1. Oral manifestation of cleido cranial displasia.

    PubMed

    Sberna, M T; De Angelis, D; Laruffa, F; Tettamanti, L; Storti, E

    2012-10-01

    Cleidocranial dysplasia (CCD) is a congenital hereditary condition caused by a dominant autosomal mutation. The orthodontic management of CCD patients is often complicated by the manifestations associated with the condition. The aim of this study was to evaluate the oral manifestations of patients suffering from CCD. The subjects underwent a thorough clinical evaluation that examined both hard and soft tissue. The subjects also underwent an X-ray examination in order to isolate possible anomalies in the stomach region related to the condition. Case 1 presented with a bilateral cross-bite, retention of deciduous teeth, presence of supernumerary teeth and agenesis. Case 2 presented with bilateral cross-bite, retention of deciduous teeth, and the presence of supernumerary teeth. The individuals in this study represent a broad range of typical CCD manifestations and confirm the close correlation between the mutation at CBFA1 level and the presence or oral-dental alterations. An early diagnosis of CCD is essential and based on clinical manifestations, x-ray evidence and genetic tests. Diagnosing the condition can be difficult in cases where there are no evident morphological signs; however, in most cases, an alteration in the rhythm of deciduous tooth loss is immediately apparent, making a closer collaboration between dentists and geneticists fundamental in achieving early diagnosis. As stated above, the timing of the treatment is crucial in establishing a correct course of treatment that involves extractions, orthodontic surgery and the use of prosthetics. PMID:23076024

  2. Dyslexia in Regular Orthographies: Manifestation and Causation

    ERIC Educational Resources Information Center

    Wimmer, Heinz; Schurz, Matthias

    2010-01-01

    This article summarizes our research on the manifestation of dyslexia in German and on cognitive deficits, which may account for the severe reading speed deficit and the poor orthographic spelling performance that characterize dyslexia in regular orthographies. An only limited causal role of phonological deficits (phonological awareness,…

  3. 40 CFR 267.72 - Manifest discrepancies.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... waste solvent substituted for waste acid, or toxic constituents not reported on the manifest or shipping... 267.72 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS FOR OWNERS AND OPERATORS OF HAZARDOUS WASTE FACILITIES OPERATING UNDER A STANDARDIZED...

  4. 40 CFR 267.72 - Manifest discrepancies.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... waste solvent substituted for waste acid, or toxic constituents not reported on the manifest or shipping... 267.72 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS FOR OWNERS AND OPERATORS OF HAZARDOUS WASTE FACILITIES OPERATING UNDER A STANDARDIZED...

  5. 40 CFR 267.72 - Manifest discrepancies.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... waste solvent substituted for waste acid, or toxic constituents not reported on the manifest or shipping... 267.72 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS FOR OWNERS AND OPERATORS OF HAZARDOUS WASTE FACILITIES OPERATING UNDER A STANDARDIZED...

  6. 40 CFR 267.72 - Manifest discrepancies.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... waste solvent substituted for waste acid, or toxic constituents not reported on the manifest or shipping... 267.72 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS FOR OWNERS AND OPERATORS OF HAZARDOUS WASTE FACILITIES OPERATING UNDER A STANDARDIZED...

  7. 19 CFR 122.75 - Complete manifest.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 19 Customs Duties 1 2010-04-01 2010-04-01 false Complete manifest. 122.75 Section 122.75 Customs Duties U.S. CUSTOMS AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Documents Required for Clearance and Permission To Depart;...

  8. 19 CFR 123.32 - Manifests.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 19 Customs Duties 1 2010-04-01 2010-04-01 false Manifests. 123.32 Section 123.32 Customs Duties U.S. CUSTOMS AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT OF THE TREASURY CUSTOMS RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through the United States § 123.32...

  9. 40 CFR 761.215 - Manifest discrepancies.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 40 Protection of Environment 31 2014-07-01 2014-07-01 false Manifest discrepancies. 761.215 Section 761.215 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES..., then write the generator's site address in the designated space for Item 5. (2) Write the name of...

  10. Rheumatic manifestations of inflammatory bowel disease.

    PubMed

    Rodríguez-Reyna, Tatiana Sofía; Martínez-Reyes, Cynthia; Yamamoto-Furusho, Jesús Kazúo

    2009-11-28

    This article reviews the literature concerning rheumatic manifestations of inflammatory bowel disease (IBD), including common immune-mediated pathways, frequency, clinical course and therapy. Musculoskeletal complications are frequent and well-recognized manifestations in IBD, and affect up to 33% of patients with IBD. The strong link between the bowel and the osteo-articular system is suggested by many clinical and experimental observations, notably in HLA-B27 transgenic rats. The autoimmune pathogenic mechanisms shared by IBD and spondyloarthropathies include genetic susceptibility to abnormal antigen presentation, aberrant recognition of self, the presence of autoantibodies against specific antigens shared by the colon and other extra-colonic tissues, and increased intestinal permeability. The response against microorganisms may have an important role through molecular mimicry and other mechanisms. Rheumatic manifestations of IBD have been divided into peripheral arthritis, and axial involvement, including sacroiliitis, with or without spondylitis, similar to idiopathic ankylosing spondylitis. Other periarticular features can occur, including enthesopathy, tendonitis, clubbing, periostitis, and granulomatous lesions of joints and bones. Osteoporosis and osteomalacia secondary to IBD and iatrogenic complications can also occur. The management of the rheumatic manifestations of IBD consists of physical therapy in combination with local injection of corticosteroids and nonsteroidal anti-inflammatory drugs; caution is in order however, because of their possible harmful effects on intestinal integrity, permeability, and even on gut inflammation. Sulfasalazine, methotrexate, azathioprine, cyclosporine and leflunomide should be used for selected indications. In some cases, tumor necrosis factor-alpha blocking agents should be considered as first-line therapy.

  11. Retinal pigment epithelial hamartoma--unusual manifestations.

    PubMed Central

    Rosenberg, P. R.; Walsh, J. B.

    1984-01-01

    Hamartoma of the retinal pigment epithelium is an uncommon tumour of young adults. We have seen 2 patients with this clinical diagnosis, both with unusual manifestations. In one patient growth of the tumour was observed over a 5-year period. In the second patient arterial-arterial anastomoses were detected at a site distal to the tumour. Images PMID:6722077

  12. Neoliberalism, Curriculum Development and Manifestations of "Creativity"

    ERIC Educational Resources Information Center

    Hakala, Juha T.; Uusikylä, Kari; Järvinen, Esa-Matti

    2015-01-01

    There is a manifest tendency for national education policy to follow global economic trends. In many Western industrialized countries, this relationship has intensified or strengthened within the last decades. The strengthening of this relationship has been seen, among other things, as evidence of the growing power of neoliberal ideology. The…

  13. Real-Life Contextual Manifestations of Wisdom

    ERIC Educational Resources Information Center

    Yang, Shih-Ying

    2008-01-01

    Wisdom pertains to managing human affairs, and it arises in highly contextualized situations. The present study aims to investigate manifestations of wisdom in real-life contexts through semi-structured interviews with 66 individuals nominated as wise persons. All nominees were ethnic Chinese from Taiwan, an East Asian country which has…

  14. 40 CFR 761.212 - Transporter compliance with the manifest.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... manifest that includes the facility's date and signature, and the Manifest Tracking Number of the new... new manifest must include all of the information required in 40 CFR 761.215(e)(1) through (6) or (f)(1... description of the rejection in the discrepancy block of the manifest, and the name, address, phone...

  15. 40 CFR 761.212 - Transporter compliance with the manifest.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... manifest that includes the facility's date and signature, and the Manifest Tracking Number of the new... new manifest must include all of the information required in 40 CFR 761.215(e)(1) through (6) or (f)(1... description of the rejection in the discrepancy block of the manifest, and the name, address, phone...

  16. 19 CFR 123.22 - In-transit manifest.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 19 Customs Duties 1 2014-04-01 2014-04-01 false In-transit manifest. 123.22 Section 123.22 Customs... CBP RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22 In-transit manifest. (a) Manifest required. A manifest in duplicate covering the in-transit merchandise which is...

  17. 40 CFR 262.54 - Special manifest requirements.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE Exports of Hazardous Waste § 262.54 Special manifest requirements. A primary exporter must comply with the manifest requirements of 40 CFR 262.20... (as defined in 40 CFR 264.72(a)) between the manifest and the shipment. A copy of the manifest...

  18. 40 CFR 262.54 - Special manifest requirements.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE Exports of Hazardous Waste § 262.54 Special manifest requirements. A primary exporter must comply with the manifest requirements of 40 CFR 262.20... (as defined in 40 CFR 264.72(a)) between the manifest and the shipment. A copy of the manifest...

  19. 40 CFR 262.54 - Special manifest requirements.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE Exports of Hazardous Waste § 262.54 Special manifest requirements. A primary exporter must comply with the manifest requirements of 40 CFR 262.20... (as defined in 40 CFR 264.72(a)) between the manifest and the shipment. A copy of the manifest...

  20. 40 CFR 262.54 - Special manifest requirements.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE Exports of Hazardous Waste § 262.54 Special manifest requirements. A primary exporter must comply with the manifest requirements of 40 CFR 262.20... (as defined in 40 CFR 264.72(a)) between the manifest and the shipment. A copy of the manifest...

  1. 40 CFR 262.54 - Special manifest requirements.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE Exports of Hazardous Waste § 262.54 Special manifest requirements. A primary exporter must comply with the manifest requirements of 40 CFR 262.20... (as defined in 40 CFR 264.72(a)) between the manifest and the shipment. A copy of the manifest...

  2. Ulnar ray deficiency: its various manifestations.

    PubMed

    Swanson, A B; Tada, K; Yonenobu, K

    1984-09-01

    Eighty-eight upper extremities of 65 patients with ulnar ray deficiency were reviewed with regard to clinical manifestations. Based on the findings, a subclassification into four types was established: type I, hypoplasia or partial defect of the ulna; type II, total defect of the ulna; type III, total or partial defect of the ulna with humeroradial synostosis; and type IV, ulnar defect with congenital amputation at the wrist. Various manifestations of deficiency were evident not only within the ulnar ray but also in other rays. Hypoplasia of the shoulder and/or proximal part of the humerus was present in some cases of types III and IV. Elbow involvement varied from functioning (type I) to acute flexion contracture (type II) to fusion (type III). In 57 hands the digits and carpal bones in the radial ray showed hypoplasia and/or defect. Central digits and carpal bones were also influenced by ulnar ray deficiency, presenting carpal bone fusion, syndactyly, and delta phalanx.

  3. Violent and criminal manifestations in dementia patients.

    PubMed

    Cipriani, Gabriele; Lucetti, Claudio; Danti, Sabrina; Carlesi, Cecilia; Nuti, Angelo

    2016-05-01

    Although the older adults have been studied as victims of violence, geriatric patients can display violent behavior. The purpose of the present review was to explore the phenomenon of criminal violations and violent acts in people with dementia. The authors used PubMed to search the MEDLINE database and other sources for original research and review articles on criminal and violent manifestation in demented patients combining the terms "criminal manifestation," "violence, aggressive behavior," "homicide," "suicide" and "homicide-suicide" together with "dementia". Possible biomarkers of violence are considered. The present review highlights the risk factors for violence in patients suffering from dementia, and reviews the literature about criminal violations and homicidal/suicidal behavior in this patient group. Geriatr Gerontol Int 2016; 16: 541-549. PMID:26460091

  4. [Cerebrotendinous xanthomatosis: physiopathology, clinical manifestations and genetics].

    PubMed

    Preiss, Yudith; Santos, José L; Smalley, Susan V; Maiz, Alberto

    2014-05-01

    Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive disease, caused by genetic deficiency of the 27-hydroxylase enzyme (encoded by CYP27A1). It plays a key role in cholesterol metabolism, especially in bile acid synthesis and in the 25-hydroxylation of vitamin D3 in the liver. Its deficiency causes reduced bile acid synthesis and tissue accumulation of cholestanol. Clinical manifestations are related to the presence of cholestanol deposits and include tendon xanthomas, premature cataracts, chronic diarrhea, progressive neurologic impairment and less frequently coronary heart disease, early onset osteoporosis and abnormalities in the optic disk and retina. An early diagnosis and treatment with quenodeoxycholic acid may prevent further complications, mainly neurological manifestations. This review summarizes cholesterol metabolism related to bile acid synthesis, physiopathology, biochemistry and treatment of cerebrotendinous xanthomatosis. PMID:25427019

  5. Clinical manifestations, diagnosis, and treatment of neurocysticercosis.

    PubMed

    Sotelo, Julio

    2011-12-01

    Neurocysticercosis (NCC) is the most frequent parasitic disease of the human brain. Modern imaging studies, CT and MRI, have defined the diagnosis and characterization of the disease. Through these studies the therapeutic approach for each case may be individualized with the aid of antihelmintics, steroids, symptomatic medicines, or surgery. The use of one or various therapeutic measures largely depends on the peculiar combination of number, location, and biological stage of lesions as well as the degree of inflammatory response to the parasites. Although there is not a typical clinical picture of NCC, epilepsy is the most frequent manifestation of parenchymal NCC, whereas hydrocephalus is the most frequent manifestation of meningeal NCC. Eradication of cysticercosis is an attainable goal by public education and sanitary improvement in endemic areas.

  6. Familial polyposis coli and its extracolonic manifestations.

    PubMed

    Cohen, S B

    1982-06-01

    A detailed clinical study of 30 families with familial polyposis coli is presented. Seven 'isolated' cases are also described. It was found that some families did not exhibit any extracolonic manifestations, but the majority of families showed various numbers of members who had these manifestations of differing types and degrees. In view of the great variability within the members of a family, polyposis coli and the Gardner syndrome are probably both produced by one pleiotropic gene. The occurrence of other neoplastic phenomena in association with polyposis coli has been considered. Many types of malignancy can occur in these patients and their families and the majority are probably fortuitous. The consistent finding of an association with medulloblastoma is such as to make this association of significance, but no reason is known for this. It is suggested that the term 'Turcot syndrome' should be used in a more restrictive manner than at present. PMID:7108915

  7. The Chronic Gastrointestinal Manifestations of Chagas Disease

    PubMed Central

    Matsuda, Nilce Mitiko; Miller, Steven M.; Evora, Paulo R. Barbosa

    2009-01-01

    Chagas disease is an infectious disease caused by the protozoan Trypanosoma cruzi. The disease mainly affects the nervous system, digestive system and heart. The objective of this review is to revise the literature and summarize the main chronic gastrointestinal manifestations of Chagas disease. The chronic gastrointestinal manifestations of Chagas disease are mainly a result of enteric nervous system impairment caused by T. cruzi infection. The anatomical locations most commonly described to be affected by Chagas disease are salivary glands, esophagus, lower esophageal sphincter, stomach, small intestine, colon, gallbladder and biliary tree. Chagas disease has also been studied in association with Helicobacter pylori infection, interstitial cells of Cajal and the incidence of gastrointestinal cancer. PMID:20037711

  8. Cardiac manifestations of idiopathic pulmonary fibrosis.

    PubMed

    Agrawal, Abhinav; Verma, Isha; Shah, Varun; Agarwal, Abhishek; Sikachi, Rutuja R

    2016-05-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, parenchymal disease of the lung with an estimated prevalence of 14-43 per 100,000. Patient usually presents with coughing and exertional dyspnea, which can lead to acute respiratory failure. IPF has been associated with various co-morbidities such as lung cancer, emphysema, obstructive sleep apnea (OSA), GERD and multiple cardiovascular consequences. The cardiovascular manifestations of IPF include pulmonary hypertension, heart failure, coronary artery disease, cardiac arrhythmias & cardiac manifestations of drugs used to treat IPF. This review will outline evidence of the association between IPF and cardiovascular conditions and attempt to provide insights into the underlying pathophysiology. We also discuss the impact of these cardiovascular diseases on patients with IPF including increased morbidity and mortality. PMID:27195188

  9. Clinical Manifestations and Diagnosis of Acromegaly

    PubMed Central

    Lugo, Gloria; Pena, Lara; Cordido, Fernando

    2012-01-01

    Acromegaly and gigantism are due to excess GH production, usually as a result of a pituitary adenoma. The incidence of acromegaly is 5 cases per million per year and the prevalence is 60 cases per million. Clinical manifestations in each patient depend on the levels of GH and IGF-I, age, tumor size, and the delay in diagnosis. Manifestations of acromegaly are varied and include acral and soft tissue overgrowth, joint pain, diabetes mellitus, hypertension, and heart and respiratory failure. Acromegaly is a disabling disease that is associated with increased morbidity and reduced life expectancy. The diagnosis is based primarily on clinical features and confirmed by measuring GH levels after oral glucose loading and the estimation of IGF-I. It has been suggested that the rate of mortality in patients with acromegaly is correlated with the degree of control of GH. Adequately treated, the relative mortality risk can be markedly reduced towards normal. PMID:22518126

  10. Cardiac manifestations of idiopathic pulmonary fibrosis

    PubMed Central

    Agrawal, Abhinav; Verma, Isha; Shah, Varun; Agarwal, Abhishek; Sikachi, Rutuja R

    2016-01-01

    Summary Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, parenchymal disease of the lung with an estimated prevalence of 14–43 per 100,000. Patient usually presents with coughing and exertional dyspnea, which can lead to acute respiratory failure. IPF has been associated with various co-morbidities such as lung cancer, emphysema, obstructive sleep apnea (OSA), GERD and multiple cardiovascular consequences. The cardiovascular manifestations of IPF include pulmonary hypertension, heart failure, coronary artery disease, cardiac arrhythmias & cardiac manifestations of drugs used to treat IPF. This review will outline evidence of the association between IPF and cardiovascular conditions and attempt to provide insights into the underlying pathophysiology. We also discuss the impact of these cardiovascular diseases on patients with IPF including increased morbidity and mortality. PMID:27195188

  11. Maxillary sinus manifestations of methamphetamine abuse.

    PubMed

    Faucett, Erynne A; Marsh, Katherine M; Farshad, Kayven; Erman, Audrey B; Chiu, Alexander G

    2015-01-01

    Methamphetamines are the second most commonly used illicit drug worldwide and cost the United States health-care system ∼$23.4 billion annually. Use of this drug affects multiple organ systems and causes a variety of clinical manifestations. Although there are commonly known sequelae of methamphetamine abuse such as "meth mouth," there is limited evidence regarding maxillary sinus manifestations. The following cases highlight the initial evaluation and management of two methamphetamine abusers with loculated purulent collections within the maxillary sinus as a result of methamphetamine abuse. Our aim was to delineate the otolaryngologic symptoms associated with the patients' methamphetamine abuse. Computed tomography and magnetic resonance imaging studies revealed loculated purulent collections within the maxillary sinus of probable odontogenic origin in both patients. Methamphetamine abuse leading to rampant caries and poor oral hygiene may predispose individuals for craniofacial infections and fluid collections. These cases illustrate the development of maxillary sinusitis and maxilla mucoceles that have been associated with methamphetamine use.

  12. Fetal cytomegalovirus infection manifesting as transient pancytopenia.

    PubMed

    Kiyokoba, Ryo; Hidaka, Nobuhiro; Sakata, Yukiyo; Hachisuga, Kazuhisa; Fukushima, Kotaro; Kato, Kiyoko

    2015-08-01

    We encountered a patient with a fetal cytomegalovirus infection manifesting as pancytopenia and thoracic hypoplasia. The fetal anemia was treated by transfusion via the umbilical cord, and did not progress after 22 weeks' gestation. The neutropenia resolved spontaneously, and only thrombocytopenia was persistent at birth. The severe thoracic hypoplasia led to pulmonary hypertension and required intensive postnatal respiratory management. Our experience suggests that pancytopenia is a possible manifestation in fetuses infected with cytomegalovirus. This may be transient, resolving spontaneously during fetal life; however, caution should be taken with blood counts, particularly platelet counts, after delivery. In addition, clinicians should carefully follow the thoracic volume in cytomegalovirus-infected fetuses and consider the possibility of postnatal severe respiratory insufficiency.

  13. [Cerebrotendinous xanthomatosis: physiopathology, clinical manifestations and genetics].

    PubMed

    Preiss, Yudith; Santos, José L; Smalley, Susan V; Maiz, Alberto

    2014-05-01

    Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive disease, caused by genetic deficiency of the 27-hydroxylase enzyme (encoded by CYP27A1). It plays a key role in cholesterol metabolism, especially in bile acid synthesis and in the 25-hydroxylation of vitamin D3 in the liver. Its deficiency causes reduced bile acid synthesis and tissue accumulation of cholestanol. Clinical manifestations are related to the presence of cholestanol deposits and include tendon xanthomas, premature cataracts, chronic diarrhea, progressive neurologic impairment and less frequently coronary heart disease, early onset osteoporosis and abnormalities in the optic disk and retina. An early diagnosis and treatment with quenodeoxycholic acid may prevent further complications, mainly neurological manifestations. This review summarizes cholesterol metabolism related to bile acid synthesis, physiopathology, biochemistry and treatment of cerebrotendinous xanthomatosis.

  14. Clinical manifestations and diagnosis of acromegaly.

    PubMed

    Lugo, Gloria; Pena, Lara; Cordido, Fernando

    2012-01-01

    Acromegaly and gigantism are due to excess GH production, usually as a result of a pituitary adenoma. The incidence of acromegaly is 5 cases per million per year and the prevalence is 60 cases per million. Clinical manifestations in each patient depend on the levels of GH and IGF-I, age, tumor size, and the delay in diagnosis. Manifestations of acromegaly are varied and include acral and soft tissue overgrowth, joint pain, diabetes mellitus, hypertension, and heart and respiratory failure. Acromegaly is a disabling disease that is associated with increased morbidity and reduced life expectancy. The diagnosis is based primarily on clinical features and confirmed by measuring GH levels after oral glucose loading and the estimation of IGF-I. It has been suggested that the rate of mortality in patients with acromegaly is correlated with the degree of control of GH. Adequately treated, the relative mortality risk can be markedly reduced towards normal. PMID:22518126

  15. [Cardiac sarcoidosis - clinical manifestation and diagnosis].

    PubMed

    Błaut-Jurkowska, Justyna; Podolec, Piotr; Olszowska, Maria

    2016-08-01

    Sarcoidosis is a multisystem inflammatory disease defined histologically by the formation of noncaseating granulomas. The etiology of sarcoidosis remains unknown. Heart involvement in the course of sarcoidosis concerns about 5% of patients. The most common manifestation of cardiac sarcoidosis are conduction abnormalities, arrhythmias and heart failure. The diagnostic algorithm includes performing a clinical history, a 12-lead electrocardiogram (ECG) and an echocardiogram. If any of the initial screening investigations yields an abnormality, diagnostics should be continue using advanced imaging techniques: cardiovascular magnetic resonance (CMR) or fluorodeoxyglucose positron emission tomography (FDG-PET). Nowadays endomyocardial biopsy is not performed routinely.The clinical picture of cardiac sarcoidosis is highly variable. Screening for cardiac sarcoidosis should be performed in all patients diagnosed with extracardiac sarcoidosis. Cardiac sarcoidosis should also be suspected in young patients without a diagnosis of sarcoidosis who present with conduction abnormalities of unknown etiology, because cardiac sarcoidosis may be the first or the only manifestation of the disease. PMID:27591449

  16. [Cardiac sarcoidosis - clinical manifestation and diagnosis].

    PubMed

    Błaut-Jurkowska, Justyna; Podolec, Piotr; Olszowska, Maria

    2016-07-01

    Sarcoidosis is a multisystem inflammatory disease defined histologically by the formation of noncaseating granulomas. The etiology of sarcoidosis remains unknown. Heart involvement in the course of sarcoidosis concerns about 5% of patients. The most common manifestation of cardiac sarcoidosis are conduction abnormalities, arrhythmias and heart failure. The diagnostic algorithm includes performing a clinical history, a 12-lead electrocardiogram (ECG) and an echocardiogram. If any of the initial screening investigations yields an abnormality, diagnostics should be continue using advanced imaging techniques: cardiovascular magnetic resonance (CMR) or fluorodeoxyglucose positron emission tomography (FDG-PET). Nowadays endomyocardial biopsy is not performed routinely.The clinical picture of cardiac sarcoidosis is highly variable. Screening for cardiac sarcoidosis should be performed in all patients diagnosed with extracardiac sarcoidosis. Cardiac sarcoidosis should also be suspected in young patients without a diagnosis of sarcoidosis who present with conduction abnormalities of unknown etiology, because cardiac sarcoidosis may be the first or the only manifestation of the disease. PMID:27590654

  17. Acute dacryocystitis: another clinical manifestation of sporotrichosis.

    PubMed

    Freitas, Dayvison Francis Saraiva; Lima, Iluska Augusta Rocha; Curi, Carolina Lemos; Jordão, Livia; Zancopé-Oliveira, Rosely Maria; Valle, Antonio Carlos Francesconi do; Galhardo, Maria Clara Gutierrez; Curi, Andre Luiz Land

    2014-04-01

    Sporotrichosis associated with exposure to domestic cats is hyperendemic in Rio de Janeiro, Brazil. A review of the clinical records at our institute revealed four patients with clinical signs of dacryocystitis and a positive conjunctival culture for Sporothrix who were diagnosed with Sporothrix dacryocystitis. Three patients were children (< 13 years of age) and one patient was an adult. Two patients reported contact with a cat that had sporotrichosis. Dacryocystitis was associated with nodular, ulcerated lesions on the face of one patient and with granulomatous conjunctivitis in two patients; however, this condition manifested as an isolated disease in another patient. All of the patients were cured of the fungal infections, but three patients had chronic dacryocystitis and one patient developed a cutaneous fistula. Sporotrichosis is usually a benign disease, but may cause severe complications when the eye and the adnexa are affected. Physicians, especially ophthalmologists in endemic areas, should be aware of the ophthalmological manifestations and complications of sporotrichosis.

  18. Acute dacryocystitis: another clinical manifestation of sporotrichosis

    PubMed Central

    Freitas, Dayvison Francis Saraiva; Lima, Iluska Augusta Rocha; Curi, Carolina Lemos; Jordão, Livia; Zancopé-Oliveira, Rosely Maria; do Valle, Antonio Carlos Francesconi; Galhardo, Maria Clara Gutierrez; Curi, Andre Luiz Land

    2013-01-01

    Sporotrichosis associated with exposure to domestic cats is hyperendemic in Rio de Janeiro, Brazil. A review of the clinical records at our institute revealed four patients with clinical signs of dacryocystitis and a positive conjunctival culture for Sporothrix who were diagnosed with Sporothrix dacryocystitis. Three patients were children (< 13 years of age) and one patient was an adult. Two patients reported contact with a cat that had sporotrichosis. Dacryocystitis was associated with nodular, ulcerated lesions on the face of one patient and with granulomatous conjunctivitis in two patients; however, this condition manifested as an isolated disease in another patient. All of the patients were cured of the fungal infections, but three patients had chronic dacryocystitis and one patient developed a cutaneous fistula. Sporotrichosis is usually a benign disease, but may cause severe complications when the eye and the adnexa are affected. Physicians, especially ophthalmologists in endemic areas, should be aware of the ophthalmological manifestations and complications of sporotrichosis. PMID:24810176

  19. 40 CFR 264.72 - Manifest discrepancies.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... limits for “empty” containers set forth in 40 CFR 261.7(b). (b) Significant differences in quantity are... exceeds the quantity limits for “empty” containers set forth in 40 CFR 261.7(b), the facility must consult... set forth in 40 CFR 261.7(b) after it has signed, dated, and returned a copy of the manifest to...

  20. Peripheral Nervous System Manifestations of Infectious Diseases

    PubMed Central

    Brizzi, Kate T.

    2014-01-01

    Infectious causes of peripheral nervous system (PNS) disease are underrecognized but potentially treatable. Heightened awareness educed by advanced understanding of the presentations and management of these infections can aid diagnosis and facilitate treatment. In this review, we discuss the clinical manifestations, diagnosis, and treatment of common bacterial, viral, and parasitic infections that affect the PNS. We additionally detail PNS side effects of some frequently used antimicrobial agents. PMID:25360209

  1. Catatonia: an unusual manifestation of Wilson's disease.

    PubMed

    Basu, Aniruddha; Thanapal, Sivakumar; Sood, Mamta; Khandelwal, Sudhir K

    2015-01-01

    Wilson's disease, characterized by abnormal copper accumulation in the human body, may present with psychiatric manifestations in about one-fifth of patients. The authors report a patient with Wilson's disease who initially presented with acute psychosis and later developed catatonic symptoms. The atypical presentation led to a delay in diagnosis and institution of appropriate treatment. Wilson's disease can be ruled out in all young patients presenting with psychiatric symptoms for the first time by screening for a Kayser-Fleischer ring.

  2. Dermatologic Extrahepatic Manifestations of Hepatitis C

    PubMed Central

    Dedania, Bhavtosh; Wu, George Y.

    2015-01-01

    Hepatitis C virus (HCV) affects millions of people worldwide, and an estimated 3.2 million people in the United States. HCV is a hepatotropic and lymphotropic virus that causes not only liver disease, but also a significant number of extrahepatic manifestations (EHMs). Up to 74% of patients affected by HCV will have HCV-related EHMs of some severity in their lifetime. The EHMs vary from simple cutaneous palpable purpura to complex lymphoproliferative disorders, including lymphomas and immune-complex deposit diseases causing local and/or systemic complications. Mixed cryoglobulinemia (MC) is manifested by multiple systemic organ involvement, mainly skin, kidney, peripheral nerves, and salivary glands, and less frequently causes widespread vasculitis and malignant lymphoma. MC affects up to 3% of HCV-infected patients with cryoglobulinemia of clinical significance, i.e. >6%. Severe disease requires immunosuppressive or plasma exchange therapy. HCV prevalence in the United States in patients with porphyria cutanea tarda (PCT) was reported to be 66%, much higher than that in general population. Therefore, all patients with PCT should be screened for HCV. The skin rash of PCT varies from large blisters to small vesicles and/or milia on the hands. Skin manifestations due to PCT usually respond to anti‐HCV treatment together with reducing skin sun exposure, avoiding triggers, having routine phlebotomy (especially for people with chronic iron overload states), and using chloroquine. Lichen planus (LP), which typically affects both the skin and oral mucosa is a chronic inflammatory disease of squamous cell origin affecting about 1% of the worldwide population. The prevalence of HCV in patients with LP varies based on geographic location. We review here the basic pathophysiology, clinical features, and management of dermatologic manifestations of HCV. PMID:26357639

  3. Cutaneous Manifestations of Common Liver Diseases

    PubMed Central

    Dogra, Sunil; Jindal, Rashmi

    2012-01-01

    Skin functions as a window to our overall health and a number of systemic diseases result in various cutaneous changes. Knowledge of these manifestations helps in suspecting an underlying systemic illness. Cutaneous abnormalities are quite common in patients with liver diseases and this article aims to focus on these dermatoses. Cutaneous manifestations seen in patients with liver disease though common are nonspecific. They can also be seen in patients without liver diseases and generally do not indicate about a specific underlying hepatic disorder. The presence of a constellation of signs and symptoms is more useful in pointing toward an underlying hepatobiliary condition. The commonest symptom in patients with liver disease is pruritus which is often protracted and disabling. Other common features include spider angiomas, palmar erythema, paper money skin, xanthelasmas, pigmentary changes, and nutritional deficiencies. In this article, first the common cutaneous manifestations that may be associated with liver disorders are discussed and then common liver diseases with their specific cutaneous findings are discussed. Cutaneous abnormalities may be the first clue to the underlying liver disease. Identifying them is crucial for early diagnosis and better management. PMID:25755383

  4. Skin Manifestations of Inflammatory Bowel Disease

    PubMed Central

    Huang, Brian L.; Chandra, Stephanie; Shih, David Quan

    2012-01-01

    Inflammatory bowel disease (IBD) is a disease that affects the intestinal tract via an inflammatory process. Patients who suffer from IBD often have diseases that affect multiple other organ systems as well. These are called extraintestinal manifestations and can be just as, if not more debilitating than the intestinal inflammation itself. The skin is one of the most commonly affected organ systems in patients who suffer from IBD. The scientific literature suggests that a disturbance of the equilibrium between host defense and tolerance, and the subsequent over-activity of certain immune pathways are responsible for the cutaneous disorders seen so frequently in IBD patients. The purpose of this review article is to give an overview of the types of skin diseases that are typically seen with IBD and their respective pathogenesis, proposed mechanisms, and treatments. These cutaneous disorders can manifest as metastatic lesions, reactive processes to the intestinal inflammation, complications of IBD itself, or side effects from IBD treatments; these can be associated with IBD via genetic linkage, common autoimmune processes, or other mechanisms that will be discussed in this article. Ultimately, it is important for healthcare providers to understand that skin manifestations should always be checked and evaluated for in patients with IBD. Furthermore, skin disorders can predate gastrointestinal symptoms and thus may serve as important clinical indicators leading physicians to earlier diagnosis of IBD. PMID:22347192

  5. Chronic polyarthritis as isolated manifestation of toxocariasis.

    PubMed

    Viola, Gabriela R; Giacomin, Maria Fernanda A; França, Camila M P; Sallum, Adriana M E; Jacob, Cristina M A; Silva, Clovis A

    2016-01-01

    Human toxocariasis is a parasitic zoonosis mainly caused by Toxocara canis or Toxocara cati and is acquired by ingestion of the parasite's embryonated eggs. Arthralgia and/or arthritis were reported in up to 17% of the cases, generally with acute duration (less than 6 weeks). However, to our knowledge, chronic polyarthritis, as the isolated presentation of Toxocara infection, was not reported. One of the 5809 patients that was followed up at our service (0.017%) had chronic polyarthritis as the single manifestation of toxocariasis and was described herein. A 3-year-old girl was referred to our service with severe painful chronic polyarthritis for a period longer than 10 weeks and morning stiffness of 30min. Dog contact exposure history in the recreational areas of neighborhood was reported. Her exams showed high levels of eosinophils in peripheral blood (29%), bone marrow aspirate revealed marked eosinophilia (32%) and Toxocara enzyme-linked immunosorbent assay (Elisa) was positive (1:1280). She was treated with paracetamol (40mg/kg/day) and thiabendazole (25mg/kg/day) for 10 days, and all manifestations reduced. After eight months of follow-up, she was on clinical and laboratorial remission. In conclusion, we described a case of chronic polyarthritis, as isolated manifestation of toxocariasis, mimicking juvenile idiopathic arthritis and leukemia. Importantly, this zoonosis should be considered in patients with arthritis and eosinophilia.

  6. Chronic polyarthritis as isolated manifestation of toxocariasis.

    PubMed

    Viola, Gabriela R; Giacomin, Maria Fernanda A; França, Camila M P; Sallum, Adriana M E; Jacob, Cristina M A; Silva, Clovis A

    2016-01-01

    Human toxocariasis is a parasitic zoonosis mainly caused by Toxocara canis or Toxocara cati and is acquired by ingestion of the parasite's embryonated eggs. Arthralgia and/or arthritis were reported in up to 17% of the cases, generally with acute duration (less than 6 weeks). However, to our knowledge, chronic polyarthritis, as the isolated presentation of Toxocara infection, was not reported. One of the 5809 patients that was followed up at our service (0.017%) had chronic polyarthritis as the single manifestation of toxocariasis and was described herein. A 3-year-old girl was referred to our service with severe painful chronic polyarthritis for a period longer than 10 weeks and morning stiffness of 30min. Dog contact exposure history in the recreational areas of neighborhood was reported. Her exams showed high levels of eosinophils in peripheral blood (29%), bone marrow aspirate revealed marked eosinophilia (32%) and Toxocara enzyme-linked immunosorbent assay (Elisa) was positive (1:1280). She was treated with paracetamol (40mg/kg/day) and thiabendazole (25mg/kg/day) for 10 days, and all manifestations reduced. After eight months of follow-up, she was on clinical and laboratorial remission. In conclusion, we described a case of chronic polyarthritis, as isolated manifestation of toxocariasis, mimicking juvenile idiopathic arthritis and leukemia. Importantly, this zoonosis should be considered in patients with arthritis and eosinophilia. PMID:27267534

  7. Ocular manifestations of systemic inflammatory diseases.

    PubMed

    Mohsenin, Amir; Huang, John J

    2012-10-01

    Inflammation of the eye is often times seen in association with systemic inflammatory diseases. Understanding the various forms of ocular involvement in these conditions is important as untreated ophthalmic involvement can lead to severe vision loss. In addition to providing a basic framework for diagnosis and treatment, this review will highlight the ocular manifestations of the following systemic inflammatory conditions: rheumatoid arthritis, systemic lupus erythematosus, Wegener's granulomatosis, Sjögren's syndrome, polyarteritisnodosa, primary antiphospholipid syndrome, Behçet's syndrome, Kawasaki disease, Cogan's syndrome and relapsing polychondritis.

  8. Morphologic manifestations of testicular and epididymal toxicity

    PubMed Central

    Vidal, Justin D; Whitney, Katharine M

    2014-01-01

    Histopathologic examination of the testis is the most sensitive means to detect effects on spermatogenesis; however, the complexity of testicular histology, interrelatedness of cell types within the testis, and long duration of spermatogenesis can make assessment of a testicular toxicant challenging. A thorough understanding of the histology and morphologic manifestations of response to injury is critical to successfully identify a testicular effect and to begin to understand the underlying mechanism of action. The basic patterns of response to xenobiotic-induced injury to the testis and epididymis are detailed and discussed. PMID:26413388

  9. Manifestation of optical activity in different materials

    NASA Astrophysics Data System (ADS)

    Konstantinova, A. F.; Golovina, T. G.; Konstantinov, K. K.

    2014-07-01

    Various manifestations of optical activity (OA) in crystals and organic materials are considered. Examples of optically active enantiomorphic and nonenantiomorphic crystals of 18 symmetry classes are presented. The OA of enantiomorphic organic materials as components of living nature (amino acids, sugars, and proteins) is analyzed. Questions related to the origin of life on earth are considered. Examples of differences in the enantiomers of drugs are shown. The consequences of replacing conventional left-handed amino acids with additionally right-handed amino acids for living organisms are indicated.

  10. Retinal manifestations of ophthalmic artery hypoperfusion.

    PubMed

    Ong, Terrence J; Paine, Mark; O'Day, Justin

    2002-08-01

    Ophthalmic artery hypoperfusion is a relatively uncommon clinical entity. This study illustrates the posterior segment findings of ophthalmic artery hypoperfusion in a series of nine patients. Colour photographs and relevant fluorescein angiograms highlighting the findings are shown. The retinal manifestations of ophthalmic artery hypoperfusion in this series of patients include midperipheral haemorrhages, dilated retinal veins, optic disk collaterals, optic disk neo-vascularization, cotton wool spots, grey intraretinal lesions, fundus pallor, optic disk swelling and choroidal infarcts. Recognition of the ophthalmic changes in this condition may lead to detection of carotid artery disease, the surgical and medical treatment of which has important bearing on patient management.

  11. Skin manifestations of chronic kidney disease.

    PubMed

    Robles-Mendez, J C; Vazquez-Martinez, O; Ocampo-Candiani, J

    2015-10-01

    Skin manifestations associated with chronic kidney disease are very common. Most of these conditions present in the end stages and may affect the patient's quality of life. Knowledge of these entities can contribute to establishing an accurate diagnosis and prognosis. Severe renal pruritus is associated with increased mortality and a poor prognosis. Nail exploration can provide clues about albumin and urea levels. Nephrogenic systemic fibrosis is a preventable disease associated with gadolinium contrast. Comorbidities, such as diabetes mellitus and secondary hyperparathyroidism, can lead to acquired perforating dermatosis and calciphylaxis, respectively. Effective and innovative treatments are available for all of these conditions.

  12. Neurologic manifestations of iron deficiency in childhood.

    PubMed

    Yager, Jerome Y; Hartfield, Dawn S

    2002-08-01

    Iron deficiency is a common disorder in pediatric patients. Although the most common manifestation is that of anemia, iron deficiency is frequently the source of a host of neurologic disorders presenting to general pediatric neurologic practices. These disorders include developmental delay, stroke, breath-holding episodes, pseudotumor cerebri, and cranial nerve palsies. Although frequent, the identification of iron deficiency as part of the differential diagnosis in these disorders is uncommon and frequently goes untreated. The purpose of the current review is to highlight what is understood regarding iron deficiency and it's underlying pathophysiology as it relates to the brain, and the association of iron deficiency with common neurologic pediatric disease.

  13. Manifest duality for partially massless higher spins

    NASA Astrophysics Data System (ADS)

    Hinterbichler, Kurt; Joyce, Austin

    2016-09-01

    In four dimensions, partially massless fields of all spins and depths possess a duality invariance akin to electric-magnetic duality. We construct metric-like gauge invariant curvature tensors for partially massless fields of all integer spins and depths, and show how the partially massless equations of motion can be recovered from first order field equations and Bianchi identities for these curvatures. This formulation displays duality in its manifestly local and covariant form, in which it acts to interchange the field equations and Bianchi identities.

  14. Systemic Sclerosis with Multiple Pulmonary Manifestations

    PubMed Central

    Suresh, Parinita; Reddy, Venkata Siva Prasad; Sharma, Tarun; Salim, Nabil Ahmed

    2016-01-01

    Systemic sclerosis (SSc) is a chronic autoimmune multisystem disorder characterized by endothelial dysfunction and fibroblast dysfunction, which results in progressive fibrosis of the skin and internal organs more frequently the lungs and gastro intestinal tract. Pulmonary involvement is common in the course of SSc, with Interstitial Lung Disease (ILD) and Pulmonary Arterial Hypertension (PAH) being the leading causes of death. Here we report, case of an elderly female patient presenting with Diffuse SSc with multiple uncommon pulmonary manifestations like ILD with Usual Interstitial Pneumonia (UIP) pattern (usually less common), PAH and right sided pleural effusion. PMID:27504339

  15. Extradigestive manifestations of IBD in pediatrics.

    PubMed

    Aloi, M; Cucchiara, S

    2009-03-01

    Inflammatory bowel diseases (IBD) are often associated with extraintestinal manifestations (EIMs), which occur in approximately one third of patients. There is only few published data on the occurrence of these manifestations in children and adolescents, so most of the data are taken by studies in adult patients. The organs most commonly affected are joints, skin, eyes and biliary tract, although nearly every organ may be involved. Some of the EIMs are clearly related to intestinal disease activity (i.e., erythema nodosum, peripheral arthritis, orofacial lesions), whereas others occur independently (i.e., pyoderma gangrenosum, anterior uveitis/iritis, ankylosing spondylitis, primary sclerosing cholangitis). Many extraintestinal disorders may be direct inflammatory and metabolic complications of the intestinal inflammation (i.e., osteoporosis, growth retardation, nephrolithiasis, ureteral obstruction, thromboembolic disease). In this review we provide an overview on the prevalence and clinical aspects of the more commonly reported EIMs of Crohn's disease and ulcerative colitis in pediatric patients, focusing on specific issues of children affected by IBD (growth failure and metabolic osteopathy). PMID:19530508

  16. CANDLE Syndrome: orodfacial manifestations and dental implications.

    PubMed

    Roberts, T; Stephen, L; Scott, C; di Pasquale, T; Naser-Eldin, A; Chetty, M; Shaik, S; Lewandowski, L; Beighton, P

    2015-01-01

    A South African girl with CANDLE Syndrome is reported with emphasis on the orodental features and dental management. Clinical manifestations included short stature, wasting of the soft tissue of the arms and legs, erythematous skin eruptions and a prominent abdomen due to hepatosplenomegaly. Generalized microdontia, confirmed by tooth measurement and osteopenia of her jaws, confirmed by digitalized radiography, were previously undescribed syndromic components. Intellectual impairment posed problems during dental intervention. The carious dental lesions and poor oral hygiene were treated conservatively under local anaesthetic. Prophylactic antibiotics were administered an hour before all procedures.Due to the nature of her general condition, invasive dental procedures were minimal. Regular follow-ups were scheduled at six monthly intervals. During this period, her overall oral health status had improved markedly.The CANDLE syndrome is a rare condition with grave complications including immunosuppression and diabetes mellitus. As with many genetic disorders, the dental manifestations are often overshadowed by other more conspicuous and complex syndromic features. Recognition of both the clinical and oral changes that occur in the CANDLE syndrome facilitates accurate diagnosis and appropriate dental management of this potentially lethal condition. PMID:26711936

  17. Cutaneous and ocular manifestations of neurocutaneous syndromes.

    PubMed

    Chernoff, Karen A; Schaffer, Julie V

    2016-01-01

    Neurocutaneous syndromes are a heterogeneous group of congenital and hereditary disorders with manifestations in the skin and the nervous system, usually together with ocular features that represent diagnostic clues and potential sources of morbidity. Dermatologists and ophthalmologists often need to work together in identifying and managing patients with these conditions; herein, we focus on classic and under-recognized neurocutaneous syndromes. We begin with autosomal dominant genodermatoses characterized by hamartomas and tumors in the skin, eyes, and central nervous system: neurofibromatosis type 1, tuberous sclerosis complex, and PTEN hamartoma-tumor syndrome. This is followed by a discussion of two mosaic disorders, Sturge-Weber syndrome and neurocutaneous melanocytosis. In addition to providing an update on clinical presentations and evaluation of patients with these conditions, we review recent insights into their pathogenesis, drawing attention to relationships among the diseases on a molecular level and implications regarding treatment. We also highlight the major features of other neurocutaneous syndromes that have ocular findings plus pigmentary, vascular, hyperkeratotic, adnexal, connective tissue, photosensitive, and inflammatory manifestations in the skin. PMID:26903185

  18. Hematologic manifestations of Helicobacter pylori infection.

    PubMed

    Campuzano-Maya, Germán

    2014-09-28

    Helicobacter pylori (H. pylori) is the most common infection in humans, with a marked disparity between developed and developing countries. Although H. pylori infections are asymptomatic in most infected individuals, they are intimately related to malignant gastric conditions such as gastric cancer and gastric mucosa-associated lymphoid tissue (MALT) lymphoma and to benign diseases such as gastritis and duodenal and gastric peptic ulcers. Since it was learned that bacteria could colonize the gastric mucosa, there have been reports in the medical literature of over 50 extragastric manifestations involving a variety medical areas of specialization. These areas include cardiology, dermatology, endocrinology, gynecology and obstetrics, hematology, pneumology, odontology, ophthalmology, otorhinolaryngology and pediatrics, and they encompass conditions with a range of clear evidence between the H. pylori infection and development of the disease. This literature review covers extragastric manifestations of H. pylori infection in the hematology field. It focuses on conditions that are included in international consensus and management guides for H. pylori infection, specifically iron deficiency, vitamin B12 (cobalamin) deficiency, immune thrombocytopenia, and MALT lymphoma. In addition, there is discussion of other conditions that are not included in international consensus and management guides on H. pylori, including auto-immune neutropenia, antiphospholipid syndrome, plasma cell dyscrasias, and other hematologic diseases. PMID:25278680

  19. Citing geospatial feature inventories with XML manifests

    NASA Astrophysics Data System (ADS)

    Bose, R.; McGarva, G.

    2006-12-01

    Today published scientific papers include a growing number of citations for online information sources that either complement or replace printed journals and books. We anticipate this same trend for cartographic citations used in the geosciences, following advances in web mapping and geographic feature-based services. Instead of using traditional libraries to resolve citations for print material, the geospatial citation life cycle will include requesting inventories of objects or geographic features from distributed geospatial data repositories. Using a case study from the UK Ordnance Survey MasterMap database, which is illustrative of geographic object-based products in general, we propose citing inventories of geographic objects using XML feature manifests. These manifests: (1) serve as a portable listing of sets of versioned features; (2) could be used as citations within the identification portion of an international geospatial metadata standard; (3) could be incorporated into geospatial data transfer formats such as GML; but (4) can be resolved only with comprehensive, curated repositories of current and historic data. This work has implications for any researcher who foresees the need to make or resolve references to online geospatial databases.

  20. Attention Deficit Hyperactivity Disorder: manifestation in adulthood.

    PubMed

    Riccio, Cynthia A; Wolfe, Monica; Davis, Brandon; Romine, Cassandra; George, Carrie; Lee, Donghyung

    2005-03-01

    The purpose of the current study was to investigate the manifestation of ADHD in adults using a combination of structured clinical interview, behavioral self-report, and a range of neuropsychological measures. Symptom criteria that are endorsed by adults with ADHD as compared to non-diagnosed adults and an adult sample with other clinical disorders tend to reflect problems with follow-through, forgetting, organization, and losing things. Notably, adults in the No Diagnosis group endorsed a higher frequency of symptoms than base rates reported elsewhere. Related to sense of time, adults with ADHD endorsed problems with meeting deadlines, not completing tasks, not planning ahead, and having a poorer sense of time significantly more frequently than adults in either the No-Diagnosis or Other Clinical Disorder group. Results highlighted the need for further research specific to the manifestation of ADHD in adulthood and the development of diagnostic criteria that take into account the differences in development as well as age-related differences in contextual demands. PMID:15708734

  1. Hematologic manifestations of Helicobacter pylori infection

    PubMed Central

    Campuzano-Maya, Germán

    2014-01-01

    Helicobacter pylori (H. pylori) is the most common infection in humans, with a marked disparity between developed and developing countries. Although H. pylori infections are asymptomatic in most infected individuals, they are intimately related to malignant gastric conditions such as gastric cancer and gastric mucosa-associated lymphoid tissue (MALT) lymphoma and to benign diseases such as gastritis and duodenal and gastric peptic ulcers. Since it was learned that bacteria could colonize the gastric mucosa, there have been reports in the medical literature of over 50 extragastric manifestations involving a variety medical areas of specialization. These areas include cardiology, dermatology, endocrinology, gynecology and obstetrics, hematology, pneumology, odontology, ophthalmology, otorhinolaryngology and pediatrics, and they encompass conditions with a range of clear evidence between the H. pylori infection and development of the disease. This literature review covers extragastric manifestations of H. pylori infection in the hematology field. It focuses on conditions that are included in international consensus and management guides for H. pylori infection, specifically iron deficiency, vitamin B12 (cobalamin) deficiency, immune thrombocytopenia, and MALT lymphoma. In addition, there is discussion of other conditions that are not included in international consensus and management guides on H. pylori, including auto-immune neutropenia, antiphospholipid syndrome, plasma cell dyscrasias, and other hematologic diseases. PMID:25278680

  2. Autoimmune Schizophrenia? Psychiatric Manifestations of Hashimoto's Encephalitis

    PubMed Central

    Alam, Maryam; Adetutu, Ebun; Thakur, Richa; Gottlich, Caleb; DeBacker, Danielle L; Marks, Lianne

    2016-01-01

    Hashimoto’s encephalitis (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), can be a debilitating manifestation of an autoimmune reaction against the thyroid that is often under-diagnosed primarily due to a lack of definitive diagnostic criteria. This is a case of a 52-year-old woman who has been diagnosed with HE after presenting with recurrent and severe psychosis in conjunction with paranoia and a thyroidopathy. Her symptoms are chronic, having first been documented as presenting 15 years prior and showing progressive exacerbation in both frequency and severity. The patient’s paranoia often manifested as delusions involving family members or close friends and consequently introduced an opportunity for harm to herself and others. She showed great conviction with self-diagnoses that were proven incorrect, resulting in occasional non-compliance. Between episodes, the patient did not show evidence of symptoms. This patient struggled with several incorrect diagnoses and treatments for several years before the correct diagnosis of HE was made and displayed extreme improvement upon corticosteroid administration. This case illustrates the importance of increasing awareness of HE as well as including HE in a differential diagnosis when any patient presents with psychosis and concurrent thyroidopathy. Hashimoto’s encephalitis follows putative characteristics of autoimmune diseases, exhibiting a higher incidence in women as compared to men, presenting with increased titers of autoantibodies, and showing dramatic amelioration when treated with corticosteroids. PMID:27672526

  3. [Ebola: characterization, history and cutaneous manifestations].

    PubMed

    Kolbach, Marianne; Carrasco-Zuber, Juan Eduardo; Vial-Letelier, Verónica

    2015-11-01

    Ebola virus (EV) is one of the most virulent human pathogens. Fruits bats are its natural reservoir, the transmission to humans is across wild animals (especially primates) and the propagation in human populations is through bodily fluid contact. The actual outbreak started in December 2013 and crossed continental borders. Up to now, there are 17,145 suspected and confirmed cases with 6,070 deaths, resulting a total case fatality rate of 35%. Clinical manifestations can be divided in 3 phases. In phase I, symptoms are similar to flu, which may appear in a range of 2 to 21 days. In phase II which occurs in over 50% of cases, visceral symptoms and mucocutaneous manifestations appear within 4 and 5 days of the onset of symptoms. The main symptoms are a macular or maculopapular non-pruritic rash, desquamation and mucosal involvement of eyes, mouth and pharynx. In phase III, recovery or death occurs. The diagnosis is made on clinical grounds, epidemiological suspicion and a positive polymerase chain reaction (PCR) test. The treatment is supportive. If there is a suspected case, it should be notified immediately and all relevant safety measures should be instituted. PMID:26757869

  4. Autoimmune Schizophrenia? Psychiatric Manifestations of Hashimoto's Encephalitis

    PubMed Central

    Alam, Maryam; Adetutu, Ebun; Thakur, Richa; Gottlich, Caleb; DeBacker, Danielle L; Marks, Lianne

    2016-01-01

    Hashimoto’s encephalitis (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), can be a debilitating manifestation of an autoimmune reaction against the thyroid that is often under-diagnosed primarily due to a lack of definitive diagnostic criteria. This is a case of a 52-year-old woman who has been diagnosed with HE after presenting with recurrent and severe psychosis in conjunction with paranoia and a thyroidopathy. Her symptoms are chronic, having first been documented as presenting 15 years prior and showing progressive exacerbation in both frequency and severity. The patient’s paranoia often manifested as delusions involving family members or close friends and consequently introduced an opportunity for harm to herself and others. She showed great conviction with self-diagnoses that were proven incorrect, resulting in occasional non-compliance. Between episodes, the patient did not show evidence of symptoms. This patient struggled with several incorrect diagnoses and treatments for several years before the correct diagnosis of HE was made and displayed extreme improvement upon corticosteroid administration. This case illustrates the importance of increasing awareness of HE as well as including HE in a differential diagnosis when any patient presents with psychosis and concurrent thyroidopathy. Hashimoto’s encephalitis follows putative characteristics of autoimmune diseases, exhibiting a higher incidence in women as compared to men, presenting with increased titers of autoantibodies, and showing dramatic amelioration when treated with corticosteroids.

  5. Thoracic manifestations of collagen vascular diseases.

    PubMed

    Capobianco, Julia; Grimberg, Alexandre; Thompson, Bruna M; Antunes, Viviane B; Jasinowodolinski, Dany; Meirelles, Gustavo S P

    2012-01-01

    Collagen vascular diseases are a diverse group of immunologically mediated systemic disorders that often lead to thoracic changes. The collagen vascular diseases that most commonly involve the lung are rheumatoid arthritis, progressive systemic sclerosis, systemic lupus erythematosus, polymyositis and dermatomyositis, mixed connective tissue disease, and Sjögren syndrome. Interstitial lung disease and pulmonary arterial hypertension are the main causes of mortality and morbidity among patients with collagen vascular diseases. Given the broad spectrum of possible thoracic manifestations and the varying frequency with which different interstitial lung diseases occur, the interpretation of thoracic images obtained in patients with collagen vascular diseases can be challenging. The task may be more difficult in the presence of treatment-related complications such as drug toxicity and infections, which are common in this group of patients. Although chest radiography is most often used for screening and monitoring of thoracic alterations, high-resolution computed tomography can provide additional information about lung involvement in collagen vascular diseases and may be especially helpful for differentiating specific disease patterns in the lung. General knowledge about the manifestations of thoracic involvement in collagen vascular diseases allows radiologists to provide better guidance for treatment and follow-up of these patients.

  6. Autoimmune Schizophrenia? Psychiatric Manifestations of Hashimoto's Encephalitis.

    PubMed

    Haider, Ali S; Alam, Maryam; Adetutu, Ebun; Thakur, Richa; Gottlich, Caleb; DeBacker, Danielle L; Marks, Lianne

    2016-01-01

    Hashimoto's encephalitis (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), can be a debilitating manifestation of an autoimmune reaction against the thyroid that is often under-diagnosed primarily due to a lack of definitive diagnostic criteria. This is a case of a 52-year-old woman who has been diagnosed with HE after presenting with recurrent and severe psychosis in conjunction with paranoia and a thyroidopathy. Her symptoms are chronic, having first been documented as presenting 15 years prior and showing progressive exacerbation in both frequency and severity. The patient's paranoia often manifested as delusions involving family members or close friends and consequently introduced an opportunity for harm to herself and others. She showed great conviction with self-diagnoses that were proven incorrect, resulting in occasional non-compliance. Between episodes, the patient did not show evidence of symptoms. This patient struggled with several incorrect diagnoses and treatments for several years before the correct diagnosis of HE was made and displayed extreme improvement upon corticosteroid administration. This case illustrates the importance of increasing awareness of HE as well as including HE in a differential diagnosis when any patient presents with psychosis and concurrent thyroidopathy. Hashimoto's encephalitis follows putative characteristics of autoimmune diseases, exhibiting a higher incidence in women as compared to men, presenting with increased titers of autoantibodies, and showing dramatic amelioration when treated with corticosteroids. PMID:27672526

  7. Dengue hemorrhagic fever: clinical manifestations and management.

    PubMed

    Kabra, S K; Jain, Y; Singhal, T; Ratageri, V H

    1999-01-01

    Dengue virus infection may remain asymptomatic or manifest as nonspecific viral infection to life threatening dengue hemorrhagic fever (DHF)/dengue shock syndrome (DSS). Patients with DHF/DSS have fever, hemorrhagic manifestations along with thrombocytopenia and hemoconcentration. Thrombocytopenia and hemoconcentration are distinguishing features between DHF/DSS and dengue fever (DF). Some patients with dengue fever may have significant bleed and mild thrombocytopenia but no hemoconcentration. These patients are labelled to have dengue fever with unusual bleeds. Laboratory findings in DHF/DSS include rising hematocrit, thrombocytopenia and transformed lymphocytes on peripheral smear. There may be increased transaminases, hyponatremia, transient increase in blood urea nitrogen and creatinine. In severe disease there may be lab evidence of dissemination intravascular coagulation. X-ray film of the chest may show pleural-effusion. Ultrasonogram of abdomen may detect thickened gall bladder wall with hepatomegaly and ascitis. In some patients there may be abnormality in electrocardiogram and echocardiogram. The diagnosis of DHF/DSS is based on typical clinical findings. For confirmation of dengue virus infection viral culture can be done on blood obtained from patients during early phase of illness. In later part of illness antibodies against dengue virus can be demonstrated by various techniques. The treatment of DF is symptomatic. For control of fever nonsteroidal anti-inflammatory drugs should be avoided. DHF/DSS are managed by intravenous fluid infusion with repeated monitoring of vital parameters and packed cell volume (PCV).

  8. [Psychiatric manifestations by prions. A narrative review].

    PubMed

    Carrillo Robles, Daniel; García Maldonado, Gerardo

    2016-01-01

    Prion diseases are a group of rare and rapidly progressive neurodegenerative conditions that may cause neuropsychiatric symptoms. This group of diseases has been described since the 18(th) century, but they were recognized decades later, when it became clear that the humans were affected by infected animals. There was controversy when the problem was attributed to a single protein with infective capacity. The common pathological process is characterized by the conversion of the normal cellular prion protein into an abnormal form. In humans, the illness has been classified as idiopathic, inherited and acquired through exposure to exogenous material containing abnormal prions. The most prominent neurological manifestation of prion diseases is the emergence of a rapidly progressive dementia, mioclonus associated with cerebellar ataxia and also extra pyramidal symptoms. Psychiatric symptoms occur in early stages of the illness and can contribute to timely diagnosis of this syndrome. Psychiatric symptoms have traditionally been grouped in three categories: affective symptoms, impaired motor function and psychotic symptoms. Such events usually occur during the prodromal period prior to the neurological manifestations and consists in the presence of social isolation, onset of delusions, irritability/aggression, visual hallucinations, anxiety and depression, and less frequent first-rank symptoms among others. Definite diagnosis requires post mortem examination. The possibility that a large number of cases may occur in the next years or that many cases have not been considered with this diagnosis is a fact. In our opinion, psychiatrists should be aware of symptoms of this disease. The main objective of this research consisted of assessing the correlation between this disturbance and neuro-psychiatric symptoms and particularly if this psychiatric manifestations integrate a clinical picture suggestive for the diagnosis of these diseases, but firstly reviewed taxonomic

  9. 19 CFR 122.48 - Air cargo manifest.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 19 Customs Duties 1 2013-04-01 2013-04-01 false Air cargo manifest. 122.48 Section 122.48 Customs... AIR COMMERCE REGULATIONS Aircraft Entry and Entry Documents; Electronic Manifest Requirements for..., and Overflying the United States § 122.48 Air cargo manifest. (a) When required. Except as provided...

  10. 19 CFR 122.48 - Air cargo manifest.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 19 Customs Duties 1 2011-04-01 2011-04-01 false Air cargo manifest. 122.48 Section 122.48 Customs... AIR COMMERCE REGULATIONS Aircraft Entry and Entry Documents; Electronic Manifest Requirements for..., and Overflying the United States § 122.48 Air cargo manifest. (a) When required. Except as provided...

  11. 40 CFR 263.21 - Compliance with the manifest.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... facility's date and signature, and the Manifest Tracking Number of the new manifest that will accompany the... shipment, and the new manifest must include all of the information required in 40 CFR 264.72(e)(1) through (6) or (f)(1) through (6) or 40 CFR 265.72(e)(1) through (6) or (f)(1) through (6). (ii) For a...

  12. 40 CFR 263.21 - Compliance with the manifest.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... facility's date and signature, and the Manifest Tracking Number of the new manifest that will accompany the... shipment, and the new manifest must include all of the information required in 40 CFR 264.72(e)(1) through (6) or (f)(1) through (6) or 40 CFR 265.72(e)(1) through (6) or (f)(1) through (6). (ii) For a...

  13. 40 CFR 263.21 - Compliance with the manifest.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... facility's date and signature, and the Manifest Tracking Number of the new manifest that will accompany the... shipment, and the new manifest must include all of the information required in 40 CFR 264.72(e)(1) through (6) or (f)(1) through (6) or 40 CFR 265.72(e)(1) through (6) or (f)(1) through (6). (ii) For a...

  14. 40 CFR 263.21 - Compliance with the manifest.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... facility's date and signature, and the Manifest Tracking Number of the new manifest that will accompany the... shipment, and the new manifest must include all of the information required in 40 CFR 264.72(e)(1) through (6) or (f)(1) through (6) or 40 CFR 265.72(e)(1) through (6) or (f)(1) through (6). (ii) For a...

  15. 40 CFR 263.21 - Compliance with the manifest.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... facility's date and signature, and the Manifest Tracking Number of the new manifest that will accompany the... shipment, and the new manifest must include all of the information required in 40 CFR 264.72(e)(1) through (6) or (f)(1) through (6) or 40 CFR 265.72(e)(1) through (6) or (f)(1) through (6). (ii) For a...

  16. 19 CFR 122.48 - Air cargo manifest.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 19 Customs Duties 1 2010-04-01 2010-04-01 false Air cargo manifest. 122.48 Section 122.48 Customs... AIR COMMERCE REGULATIONS Aircraft Entry and Entry Documents; Electronic Manifest Requirements for..., and Overflying the United States § 122.48 Air cargo manifest. (a) When required. Except as provided...

  17. 49 CFR 172.205 - Hazardous waste manifest.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ...) hazardous waste manifest (manifest) is prepared in accordance with 40 CFR 262.20 and is signed, carried, and... in accordance with 40 CFR 263.22. (5) Before accepting hazardous waste from a rail transporter, a non.... (h) A hazardous waste manifest required by 40 CFR part 262, containing all of the...

  18. Articular and abarticular manifestations in type 2 diabetes mellitus

    PubMed Central

    Abourazzak, Fatima Ezzahra; Akasbi, Nessrine; Houssaini, Ghita Sqalli; Bazouti, Sabah; Bensbaa, Salma; Hachimi, Hicham; Ajdi, Farida; Harzy, Taoufik

    2014-01-01

    Objective Diabetes mellitus (DM), a worldwide high-prevalence disease, is associated with a large variety of rheumatic manifestations. It affects the connective tissues in many ways and causes alterations in the periarticular and the musculoskeletal systems. In most cases, these manifestations are associated with functional disability and pain, affecting the quality of life of the diabetic patient. The aim of our study is to review the different articular and abarticular manifestations in diabetic patients and the associated factors of these rheumatic manifestations. Material and Methods A cross-sectional study that includes all patients suffering from type 2 DM who present with articular or abarticular manifestations. Results We included 116 diabetic patients presenting with articular or abarticular manifestations. Our study showed four important findings. First, a large variety of articular and abarticular manifestations were present in patients with type 2 DM. Second, osteoarthritis (OA) of the knee was the most frequent articular manifestations. It was seen in 49% of our patients. Third, the most common manifestations in diabetic Moroccan patients were carpal tunnel syndrome (CTS), adhesive capsulitis of the shoulder, and diabetic cheiroarthropathy (29%, 23%, and 16%, respectively). Fourth, there was a significant association between vascular complications and the development of articular and abarticular manifestations. Conclusion This study shows that the articular and abarticular manifestations in diabetic Moroccan patients are dominated by CTS, adhesive capsulitis of the shoulder, and diabetic cheiroarthropathy, with a significant association between vascular complications and the development of some of these manifestations. PMID:27708897

  19. 49 CFR 172.205 - Hazardous waste manifest.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ...) hazardous waste manifest (manifest) is prepared in accordance with 40 CFR 262.20 and is signed, carried, and... in accordance with 40 CFR 263.22. (5) Before accepting hazardous waste from a rail transporter, a non.... (h) A hazardous waste manifest required by 40 CFR part 262, containing all of the...

  20. 40 CFR 262.23 - Use of the manifest.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... returned to the generator by the designated facility (following the procedures of 40 CFR 264.72(f) or 265...) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.23 Use of the manifest. (a) The generator must: (1) Sign the manifest certification by hand; and (2) Obtain the handwritten signature of...

  1. 40 CFR 262.23 - Use of the manifest.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... returned to the generator by the designated facility (following the procedures of 40 CFR 264.72(f) or 265...) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.23 Use of the manifest. (a) The generator must: (1) Sign the manifest certification by hand; and (2) Obtain the handwritten signature of...

  2. 40 CFR 262.23 - Use of the manifest.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... returned to the generator by the designated facility (following the procedures of 40 CFR 264.72(f) or 265...) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.23 Use of the manifest. (a) The generator must: (1) Sign the manifest certification by hand; and (2) Obtain the handwritten signature of...

  3. 40 CFR 262.23 - Use of the manifest.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... returned to the generator by the designated facility (following the procedures of 40 CFR 264.72(f) or 265...) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.23 Use of the manifest. (a) The generator must: (1) Sign the manifest certification by hand; and (2) Obtain the handwritten signature of...

  4. 40 CFR 262.23 - Use of the manifest.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... returned to the generator by the designated facility (following the procedures of 40 CFR 264.72(f) or 265...) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.23 Use of the manifest. (a) The generator must: (1) Sign the manifest certification by hand; and (2) Obtain the handwritten signature of...

  5. 40 CFR 262.24 - Use of the electronic manifest.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... compliance with 49 CFR 177.817, a generator originating an electronic manifest must also provide the initial... (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.24 Use of the electronic... requirement in these regulations for a generator to keep or retain a copy of each manifest is satisfied...

  6. 49 CFR 176.30 - Dangerous cargo manifest.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 49 Transportation 2 2012-10-01 2012-10-01 false Dangerous cargo manifest. 176.30 Section 176.30... Requirements § 176.30 Dangerous cargo manifest. (a) The carrier, its agents, and any person designated for this purpose by the carrier or agents shall prepare a dangerous cargo manifest, list, or stowage plan....

  7. 49 CFR 176.30 - Dangerous cargo manifest.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 49 Transportation 2 2010-10-01 2010-10-01 false Dangerous cargo manifest. 176.30 Section 176.30... Requirements § 176.30 Dangerous cargo manifest. (a) The carrier, its agents, and any person designated for this purpose by the carrier or agents shall prepare a dangerous cargo manifest, list, or stowage plan....

  8. 49 CFR 176.30 - Dangerous cargo manifest.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 49 Transportation 2 2011-10-01 2011-10-01 false Dangerous cargo manifest. 176.30 Section 176.30... Requirements § 176.30 Dangerous cargo manifest. (a) The carrier, its agents, and any person designated for this purpose by the carrier or agents shall prepare a dangerous cargo manifest, list, or stowage plan....

  9. 49 CFR 176.30 - Dangerous cargo manifest.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 49 Transportation 2 2013-10-01 2013-10-01 false Dangerous cargo manifest. 176.30 Section 176.30... Requirements § 176.30 Dangerous cargo manifest. (a) The carrier, its agents, and any person designated for this purpose by the carrier or agents must prepare a dangerous cargo manifest, list, or stowage plan....

  10. 49 CFR 176.30 - Dangerous cargo manifest.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 49 Transportation 2 2014-10-01 2014-10-01 false Dangerous cargo manifest. 176.30 Section 176.30... Requirements § 176.30 Dangerous cargo manifest. (a) The carrier, its agents, and any person designated for this purpose by the carrier or agents must prepare a dangerous cargo manifest, list, or stowage plan....

  11. 49 CFR 172.205 - Hazardous waste manifest.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ...) hazardous waste manifest (manifest) is prepared in accordance with 40 CFR 262.20 and is signed, carried, and... in accordance with 40 CFR 263.22. (5) Before accepting hazardous waste from a rail transporter, a non.... (h) A hazardous waste manifest required by 40 CFR part 262, containing all of the...

  12. 49 CFR 172.205 - Hazardous waste manifest.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ...) hazardous waste manifest (manifest) is prepared in accordance with 40 CFR 262.20 and is signed, carried, and... in accordance with 40 CFR 263.22. (5) Before accepting hazardous waste from a rail transporter, a non.... (h) A hazardous waste manifest required by 40 CFR part 262, containing all of the...

  13. Brucella arteritis: clinical manifestations, treatment, and prognosis.

    PubMed

    Herrick, Jesica A; Lederman, Robert J; Sullivan, Brigit; Powers, John H; Palmore, Tara N

    2014-06-01

    Brucellosis is the most common bacterial zoonosis, and causes a considerable burden of disease in endemic countries. Cardiovascular involvement is the main cause of mortality due to infection with Brucella spp, and most commonly manifests as endocarditis, peripheral and cerebrovascular aneurysms, or arterial and venous thromboses. We report a case of brucellosis presenting as bacteraemia and aortic endarteritis 18 years after the last known exposure to risk factors for brucella infection. The patient was treated with doxycycline, rifampicin, and gentamicin, and underwent surgical repair of a penetrating aortic ulcer, with a good clinical recovery. We review the signs and symptoms, diagnostic approach, prognosis, and treatment of brucella arteritis. We draw attention to the absence of consensus about the optimum therapy for vascular brucellosis, and the urgent need for additional studies and renewed scientific interest in this major pathogen. PMID:24480149

  14. Electrode and device problems: manifestation and management.

    PubMed

    Twomey, T; Archbold, S

    1997-11-01

    A total of 119 children were implanted with the Nucleus 22 implant on the Nottingham program by March 1996. Twenty-five (i.e. 21%) of these had an electrophysiologically confirmed fault on at least 1 channel and 6 (i.e. 5%) had experienced total device failure. How these problems were first manifested and what the subsequent effects were on the child and family were determined by means of questionnaires and detailed examination of clinical notes. Our findings indicated that 76% of all internal device faults were initially detected in tuning or electrophysiological measurement. All parents expressed deep anxiety about the threat of device failure; cases of total failure occurring over a long period of time resulted in the greatest distress and effect on the family. Recommendations are made for companies to assist clinics in minimizing such effects.

  15. Skin manifestations of hormone-secreting tumors.

    PubMed

    Jabbour, Serge A

    2010-01-01

    Endocrine and metabolic diseases, besides affecting other organs, can result in changes in cutaneous function and morphology and can lead to a complex symptomatology. Dermatologists may see some of these skin lesions first, either before the endocrinologist, or even after the internist or specialist has missed the right diagnosis. Because some skin lesions might reflect a life-threatening endocrine or metabolic disorder, identifying the underlying disorder is very important, so that patients can receive corrective rather than symptomatic treatment. In this issue, we will review various hormone-secreting tumors, including pituitary disorders (Cushing's syndrome and acromegaly), hyperthyroidism, glucagonoma, carcinoid syndrome, mastocytosis, and hyperandrogenism. We will focus on clinical manifestations, mainly cutaneous, followed by a brief discussion on how to make the diagnosis of each condition in addition to treatment options. PMID:21054708

  16. Clinical manifestations and diagnosis of fibromyalgia.

    PubMed

    Bennett, Robert M

    2009-05-01

    Since the publication of the American College of Rheumatology Classification Criteria for Fibromyalgia 18 years ago, there have been an ever-increasing number of research articles and reviews. From the National Library of Medicine alone there are more than 10,000 articles related to fibromyalgia. The major clinical manifestations of fibromyalgia have not changed, but their prevalence, associations, relative importance to the patient, and scientific underpinnings are increasingly better understood. This article provides an update on fibromyalgia symptomatology and looks at issues that need to be considered in the development of updated diagnostic guidelines. There is still no gold standard for making a diagnosis of fibromyalgia, but there is an increasing consensus for the development of new guidelines for diagnosis that modifies the currently proscribed tender point evaluation.

  17. Coronary Artery Manifestations of Fibromuscular Dysplasia

    PubMed Central

    Michelis, Katherine C.; Olin, Jeffrey W.; Kadian-Dodov, Daniella; d’Escamard, Valentina; Kovacic, Jason C.

    2015-01-01

    Fibromuscular dysplasia (FMD) involving the coronary arteries is an uncommon but important condition that can present as acute coronary syndrome, left ventricular dysfunction, or potentially sudden cardiac death. Although the classic angiographic “string of beads” that may be observed in renal artery FMD does not occur in coronary arteries, potential manifestations include spontaneous coronary artery dissection, distal tapering or long, smooth narrowing that may represent dissection, intramural hematoma, spasm, or tortuosity. Importantly, FMD must be identified in at least one other noncoronary arterial territory to attribute any coronary findings to FMD. Although there is limited evidence to guide treatment, many lesions heal spontaneously; thus, a conservative approach is generally preferred. The etiology is poorly understood, but there are ongoing efforts to better characterize FMD and define its genetic and molecular basis. This report reviews the clinical course of FMD involving the coronary arteries and provides guidance for diagnosis and treatment strategies. PMID:25190240

  18. Coronary artery manifestations of fibromuscular dysplasia.

    PubMed

    Michelis, Katherine C; Olin, Jeffrey W; Kadian-Dodov, Daniella; d'Escamard, Valentina; Kovacic, Jason C

    2014-09-01

    Fibromuscular dysplasia (FMD) involving the coronary arteries is an uncommon but important condition that can present as acute coronary syndrome, left ventricular dysfunction, or potentially sudden cardiac death. Although the classic angiographic "string of beads" that may be observed in renal artery FMD does not occur in coronary arteries, potential manifestations include spontaneous coronary artery dissection, distal tapering or long, smooth narrowing that may represent dissection, intramural hematoma, spasm, or tortuosity. Importantly, FMD must be identified in at least one other noncoronary arterial territory to attribute any coronary findings to FMD. Although there is limited evidence to guide treatment, many lesions heal spontaneously; thus, a conservative approach is generally preferred. The etiology is poorly understood, but there are ongoing efforts to better characterize FMD and define its genetic and molecular basis. This report reviews the clinical course of FMD involving the coronary arteries and provides guidance for diagnosis and treatment strategies.

  19. Cerebellar Stroke-manifesting as Mania

    PubMed Central

    Jagadesan, Venkatesan; Thiruvengadam, Kannapiran R.; Muralidharan, Rengarajalu

    2014-01-01

    Secondary mania resulting from cerebral Cortex are described commonly. But secondary mania produced by cerebellar lesions are relatively uncommon. This case report describes a patient who developed cerebellar stoke and manic features simultaneously. 28 years old male developed giddiness and projectile vomiting. Then he would lie down for about an hour only to find that he could not walk. He became quarrelsome. His Psycho motor activities and speech were increased. He was euphoric and was expressing grandiose ideas. Bender Gestalt Test showed signs of organicity. Score in Young mania relating scale was 32; productivity was low in Rorschach. Neurological examination revealed left cerebellar signs like ataxia and slurring of speech. Computed tomography of brain showed left cerebellar infarct. Relationship between Psychiatric manifestations and cerebellar lesion are discussed. PMID:25035567

  20. Cerebellar Stroke-manifesting as Mania.

    PubMed

    Jagadesan, Venkatesan; Thiruvengadam, Kannapiran R; Muralidharan, Rengarajalu

    2014-07-01

    Secondary mania resulting from cerebral Cortex are described commonly. But secondary mania produced by cerebellar lesions are relatively uncommon. This case report describes a patient who developed cerebellar stoke and manic features simultaneously. 28 years old male developed giddiness and projectile vomiting. Then he would lie down for about an hour only to find that he could not walk. He became quarrelsome. His Psycho motor activities and speech were increased. He was euphoric and was expressing grandiose ideas. Bender Gestalt Test showed signs of organicity. Score in Young mania relating scale was 32; productivity was low in Rorschach. Neurological examination revealed left cerebellar signs like ataxia and slurring of speech. Computed tomography of brain showed left cerebellar infarct. Relationship between Psychiatric manifestations and cerebellar lesion are discussed. PMID:25035567

  1. Clinical manifestations of pediatric idiopathic hypercalciuria.

    PubMed

    Lau, Keith K

    2009-01-01

    Idiopathic hypercalciuria (IH) is a common metabolic disorder in children with protean manifestations, many of which can mimic other common pediatric diseases. Reports in the medical literature describe children with IH presenting with a wide array of calculi and non-calculi related clinical symptoms such as hematuria, urinary tract infections (UTIs), urgency, urinary incontinence and recurrent abdominal pain. Many of these symptom complexes have been only loosely associated with IH with no definite established causal relationship. Due to the common nature of IH and the varied clinical features attributed to it, it is of utmost importance for health-care professionals to be aware of these; this will facilitate early and appropriate investigations and prompt institution of therapy to avoid long-term morbidity.

  2. Pulmonary manifestations of inflammatory bowel disease

    PubMed Central

    Majewski, Sebastian

    2015-01-01

    Bronchopulmonary signs and symptoms are examples of variable extraintestinal manifestations of the inflammatory bowel diseases (IBD). These complications of Crohn's disease (CD) and ulcerative colitis (UC) seem to be underrecognized by both pulmonary physicians and gastroenterologists. The objective of the present review was to gather and summarize information on this particular matter, on the basis of available up-to-date literature. Tracheobronchial involvement is the most prevalent respiratory presentation, whereas IBD-related interstitial lung disease is less frequent. Latent and asymptomatic pulmonary involvement is not unusual. Differential diagnosis should always consider infections (mainly tuberculosis) and drug-induced lung pathology. The common link between intestinal disease and lung pathology is unknown, but many hypotheses have been proposed. It is speculated that environmental pollution, common immunological mechanisms and predisposing genetic factors may play a role. PMID:26788078

  3. Natural curvature for manifest T-duality

    NASA Astrophysics Data System (ADS)

    Poláček, Martin; Siegel, Warren

    2014-01-01

    We reformulate the manifestly T-dual description of the massless sector of the closed bosonic string, directly from the geometry associated with the (left and right) affine Lie algebra of the coset space Poincaré/Lorentz. This construction initially doubles not only the (spacetime) coordinates for translations but also those for Lorentz transformations (and their "dual"). As a result, the Lorentz connection couples directly to the string (as does the vielbein), rather than being introduced ad hoc to the covariant derivative as previously. This not only reproduces the old definition of T-dual torsion, but automatically gives a general, covariant definition of T-dual curvature (but still with some undetermined connections).

  4. Patent foramen ovale: clinical manifestations and treatment.

    PubMed

    Kedia, Gautam; Tobis, Jonathan; Lee, Michael S

    2008-01-01

    A persistent patent foramen ovale produces an intermittent intra-atrial right-to-left shunt and occurs in approximately 25% of the general population. Although the vast majority of people with patent foramen ovale are asymptomatic, a patent foramen ovale is believed to act as a pathway for chemicals or thrombus that can result in a variety of clinical manifestations, including stroke, migraine headache, decompression sickness, high-altitude pulmonary edema, and platypnea-orthodeoxia syndrome. The optimal management of patients with patent foramen ovale who experience cryptogenic stroke is unclear. Percutaneous closure appears to have a low risk profile and has been considered in high-risk patients who are not candidates for randomized clinical trials. Randomized clinical trials that are underway should help define the best management of patent foramen ovale, as well as the true safety and efficacy of percutaneous closure devices.

  5. Cerebellar hemangioblastoma manifesting as hearing disturbance.

    PubMed

    Amano, Toshiyuki; Tokunaga, So; Shono, Tadahisa; Mizoguchi, Masahiro; Matsumoto, Kenichi; Yoshida, Fumiaki; Sasaki, Tomio

    2009-09-01

    A 49-year-old man presented with a rare case of cerebellar hemangioblastoma manifesting as only hearing disturbance. He had suffered from hearing difficulty in the right ear for a few months. Magnetic resonance imaging revealed a cystic mass lesion with an internal fluid level and surrounding flow voids in the right cerebellopontine (CP) angle. Cerebral angiography disclosed a vascular-rich tumor fed by both the superior cerebellar and anterior inferior cerebellar arteries. En bloc resection of the tumor was planned under a preoperative diagnosis of cerebellar hemangioblastoma. The tumor protruded into the CP cistern and compressed cranial nerve VIII. The feeding arteries were meticulously coagulated and the tumor was successfully removed. The histological diagnosis was hemangioblastoma. After the operation, the patient's hearing acuity improved dramatically. Cerebellar hemangioblastoma should be considered in the differential diagnosis of CP angle tumors associated with hearing disturbance.

  6. Gastrointestinal manifestations in myotonic muscular dystrophy

    PubMed Central

    Bellini, Massimo; Biagi, Sonia; Stasi, Cristina; Costa, Francesco; Mumolo, Maria Gloria; Ricchiuti, Angelo; Marchi, Santino

    2006-01-01

    Myotonic dystrophy (MD) is characterized by myotonic phenomena and progressive muscular weakness. Involvement of the gastrointestinal tract is frequent and may occur at any level. The clinical manifestations have previously been attributed to motility disorders caused by smooth muscle damage, but histologic evidence of alterations has been scarce and conflicting. A neural factor has also been hypothesized. In the upper digestive tract, dysphagia, heartburn, regurgitation and dyspepsia are the most common complaints, while in the lower tract, abdominal pain, bloating and changes in bowel habits are often reported. Digestive symptoms may be the first sign of dystrophic disease and may precede the musculo-skeletal features. The impairment of gastrointestinal function may be sometimes so gradual that the patients adapt to it with little awareness of symptoms. In such cases routine endoscopic and ultrasonographic evaluations are not sufficient and targeted techniques (electrogastrography, manometry, electromyography, functional ultrasonography, scintigraphy, etc.) are needed. There is a low correlation between the degree of skeletal muscle involvement and the presence and severity of gastrointestinal disturbances whereas a positive correlation with the duration of the skeletal muscle disease has been reported. The drugs recommended for treating the gastrointestinal complaints such as prokinetic, anti-dyspeptic drugs and laxatives, are mainly aimed at correcting the motility disorders. Gastrointestinal involvement in MD remains a complex and intriguing condition since many important problems are still unsolved. Further studies concentrating on genetic aspects, early diagnostic techniques and the development of new therapeutic strategies are needed to improve our management of the gastrointestinal manifestations of MD. PMID:16609987

  7. Musculoskeletal manifestations in patients with malignant disease.

    PubMed

    Gheita, Tamer A; Ezzat, Yasser; Sayed, Safaa; El-Mardenly, Ghada; Hammam, Waleed

    2010-02-01

    To detect and describe the incidence of musculoskeletal manifestations in different malignant diseases as well as their relation to the treatment received whether by chemotherapy or radiation therapy. Sixty patients with different malignant diseases were included in this study, 45 with solid tumors and 15 patients with hematological malignancy. The mean age was 46.55 +/- 11.04 years and the mean disease duration was 2 +/- 0.75 years. The patients were fully examined for any rheumatologic involvement, laboratory investigations were performed as well as dual energy X-ray absorptiometry study for bone densitometry. Treatment strategies were assessed including the chemotherapeutics, radiation therapy, and/or surgery. Myalgias and arthralgias were the most frequent followed by flexor tenosynovitis, frozen shoulder, and fibromyalgia syndrome. Hypertrophic osteoarthropathy was seen in five patients, cutaneous vasculitis in two patients as well as arthritis. Osteonecrosis was present in one of the lunate carpal bones of a patient with non-Hodgkin's lymphoma (1.67%) and receiving high dose steroids. Rheumatoid factor was positive in four patients, three of which had hepatitis C virus positivity and cryoglobulins. Anti-neutrophil cytoplasmic antibody was negative in all the studied patients. The bone mineral density was significantly reduced in the patients with malignancy compared to the control. Mild to moderate osteoporosis was present, being more evident in the spine and forearm. The bone loss was higher in those with solid tumors and even more obvious in those receiving aromatase inhibitors. Musculoskeletal manifestations occurring during malignancies and following the treatment represent a significant percentage of symptoms and signs which may raise a clue to differential diagnosis.

  8. Vaccines for viral diseases with dermatologic manifestations.

    PubMed

    Brentjens, Mathijs H; Yeung-Yue, Kimberly A; Lee, Patricia C; Tyring, Stephen K

    2003-04-01

    Vaccines against infectious diseases have been available since the 1800s, when an immunization strategy against smallpox developed by Jenner gained wide acceptance. Until recently, the only vaccination strategies available involved the use of protein-based, whole killed, and attenuated live virus vaccines. These strategies have led to the development of effective vaccines against a variety of diseases with primary or prominent cutaneous manifestations. Effective and safe vaccines now used worldwide include those directed against measles and rubella (now commonly used together with a mumps vaccine as the trivalent MMR), chickenpox, and hepatitis B. The eradication of naturally occurring smallpox remains one of the greatest successes in the history of modern medicine, but stockpiles of live smallpox exist in the United States and Russia. Renewed interest in the smallpox vaccine reflects concerns about a possible bioterrorist threat using this virus. Yellow fever is a hemorrhagic virus endemic to tropical areas of South America and Africa. An effective vaccine for this virus has existed since 1937, and it is used widely in endemic areas of South America, and to a lesser extent in Africa. This vaccine is recommended once every 10 years for people who are traveling to endemic areas. Advances in immunology have led to a greater understanding of immune system function in viral diseases. Progress in genetics and molecular biology has allowed researchers to design vaccines with novel mechanisms of action (eg, DNA, vector, and VLP vaccines). Vaccines have also been designed to specifically target particular viral components, allowing for stimulation of various arms of the immune system as desired. Ongoing research shows promise in prophylactic and therapeutic vaccination for viral infections with cutaneous manifestations. Further studies are necessary before vaccines for HSV, HPV, and HIV become commercially available.

  9. Vaccines for viral diseases with dermatologic manifestations.

    PubMed

    Brentjens, Mathijs H; Yeung-Yue, Kimberly A; Lee, Patricia C; Tyring, Stephen K

    2003-04-01

    Vaccines against infectious diseases have been available since the 1800s, when an immunization strategy against smallpox developed by Jenner gained wide acceptance. Until recently, the only vaccination strategies available involved the use of protein-based, whole killed, and attenuated live virus vaccines. These strategies have led to the development of effective vaccines against a variety of diseases with primary or prominent cutaneous manifestations. Effective and safe vaccines now used worldwide include those directed against measles and rubella (now commonly used together with a mumps vaccine as the trivalent MMR), chickenpox, and hepatitis B. The eradication of naturally occurring smallpox remains one of the greatest successes in the history of modern medicine, but stockpiles of live smallpox exist in the United States and Russia. Renewed interest in the smallpox vaccine reflects concerns about a possible bioterrorist threat using this virus. Yellow fever is a hemorrhagic virus endemic to tropical areas of South America and Africa. An effective vaccine for this virus has existed since 1937, and it is used widely in endemic areas of South America, and to a lesser extent in Africa. This vaccine is recommended once every 10 years for people who are traveling to endemic areas. Advances in immunology have led to a greater understanding of immune system function in viral diseases. Progress in genetics and molecular biology has allowed researchers to design vaccines with novel mechanisms of action (eg, DNA, vector, and VLP vaccines). Vaccines have also been designed to specifically target particular viral components, allowing for stimulation of various arms of the immune system as desired. Ongoing research shows promise in prophylactic and therapeutic vaccination for viral infections with cutaneous manifestations. Further studies are necessary before vaccines for HSV, HPV, and HIV become commercially available. PMID:12757257

  10. Uncommon Manifestations of Intervertebral Disk Pathologic Conditions.

    PubMed

    Diehn, Felix E; Maus, Timothy P; Morris, Jonathan M; Carr, Carrie M; Kotsenas, Amy L; Luetmer, Patrick H; Lehman, Vance T; Thielen, Kent R; Nassr, Ahmad; Wald, John T

    2016-01-01

    Beyond the familiar disk herniations with typical clinical features, intervertebral disk pathologic conditions can have a wide spectrum of imaging and clinical manifestations. The goal of this review is to illustrate and discuss unusual manifestations of intervertebral disk pathologic conditions that radiologists may encounter, including disk herniations in unusual locations, those with atypical imaging features, and those with uncommon pathophysiologic findings. Examples of atypical disk herniations presented include dorsal epidural, intradural, symptomatic thoracic (including giant calcified), extreme lateral (retroperitoneal), fluorine 18 fluorodeoxyglucose-avid, acute intravertebral (Schmorl node), and massive lumbar disk herniations. Examples of atypical pathophysiologic conditions covered are discal cysts, fibrocartilaginous emboli to the spinal cord, tiny calcified disks or disk-level spiculated osteophytes causing spinal cerebrospinal fluid (CSF) leak and intracranial hypotension, and pediatric acute calcific discitis. This broad gamut of disease includes a variety of sizes of disk pathologic conditions, from the tiny (eg, the minuscule calcified disks causing high-flow CSF leaks) to the extremely large (eg, giant calcified thoracic intradural disk herniations causing myelopathy). A spectrum of clinical acuity is represented, from hyperacute fibrocartilaginous emboli causing spinal cord infarct, to acute Schmorl nodes, to chronic intradural herniations. The entities included are characterized by a range of clinical courses, from the typically devastating cord infarct caused by fibrocartilaginous emboli, to the usually spontaneously resolving pediatric acute calcific discitis. Several conditions have important differential diagnostic considerations, and others have relatively diagnostic imaging findings. The pathophysiologic findings are well understood for some of these entities and poorly defined for others. Radiologists' knowledge of this broad scope of

  11. Giant-cell arteritis without cranial manifestations

    PubMed Central

    de Boysson, Hubert; Lambert, Marc; Liozon, Eric; Boutemy, Jonathan; Maigné, Gwénola; Ollivier, Yann; Ly, Kim; Manrique, Alain; Bienvenu, Boris; Aouba, Achille

    2016-01-01

    Abstract Diagnosis of giant-cell arteritis (GCA) is challenging in the absence of cardinal cranial symptoms/signs. We aimed to describe the clinical presentation, diagnostic process, and disease course of GCA patients without cranial symptoms, and to compare them to those of patients with typical cranial presentation. In this retrospective multicenter study, we enrolled patients with GCA who satisfied at least 3 of the 5 American College of Rheumatology criteria for GCA, or 2 criteria associated with contributory vascular biopsy other than temporal artery biopsy or with demonstration of large-vessel involvement; underwent iconographic evaluation of large arterial vessels (aortic CT scan or a positron emission tomography with 18F-fluorodeoxyglucose combined with computed tomography (FDG-PET/CT) scan or cardiac echography combined with a large-vessel Doppler) at diagnosis. We divided the cohort into 2 groups, distinguishing between patients without cranial symptoms/signs (i.e., headaches, clinical temporal artery anomaly, jaw claudication, ophthalmologic symptoms) and those with cranial symptoms/signs. In the entire cohort of 143 patients, all of whom underwent vascular biopsy and vascular imaging, we detected 31 (22%) patients with no cranial symptoms/signs. In the latter, diagnosis was biopsy proven in an arterial sample in 23 cases (74% of patients, on a temporal site in 20 cases and on an extratemporal site in 3). One-third of these 31 patients displayed extracranial symptoms/signs whereas the remaining two-thirds presented only with constitutional symptoms and/or inflammatory laboratory test results. Compared to the 112 patients with cardinal cranial clinical symptoms/signs, patients without cranial manifestations displayed lower levels of inflammatory laboratory parameters (C-reactive level: 68 [9–250] mg/L vs 120 [3–120] mg/L; P < 0.01), highest rate of aorta and aortic branch involvement identified (19/31 (61%) vs 42/112 (38%); P = 0.02) and also

  12. Biochemical Manifestation of HIV Lipodystrophy Syndrome

    PubMed Central

    Ihenetu, Kenneth; Mason, Darius

    2012-01-01

    Objectives Highly active anti-retroviral therapy (HAART), including protease inhibitors (PI) have led to dramatic improvements in the quality and quantity of life in patients with acquired immunodeficiency syndrome (AIDS). However, a significant number of AIDS patients on HAART develop characteristic changes in body fat redistribution referred to as lipodystrophy syndrome (LDS). Features of LDS include hypertrophy in the neck fat pad (buffalo hump), increased fat in the abdominal region (protease paunch), gynecomastia and loss of fat in the mid-face and extremities. Methods The aim of this paper is to review the current knowledge regarding this syndrome. This article reviews the published investigations on biochemical manifestation of HIV lipodystrophy syndrome. Results It is estimated that approximately 64% of patients treated with PI will experience this syndrome. Biochemically, these patients have increased triglycerides (Trig), total cholesterol (TC), low-density lipoprotein-cholesterol (LDL-C) and extremely low high-density lipoprotein-cholesterol (HDL-C). Conclusions and Public Health Implications It is hoped that awareness of this syndrome would aid in early diagnosis and better patient management, possibly leading to a lower incidence of cardiovascular complications among these patients.

  13. Clinical manifestations and management of Gaucher disease

    PubMed Central

    Linari, Silvia; Castaman, Giancarlo

    2015-01-01

    Summary Gaucher disease is a rare multi-systemic metabolic disorder caused by the inherited deficiency of the lysosomal enzyme β-glucocerebrosidase, which leads to the accumulation of its normal substrate, glucocerebroside, in tissue macrophages with damage to haematological, visceral and bone systems. Anaemia, thrombocytopenia, enlargement of liver and/or spleen, skeletal abnormalities (osteopenia, lytic lesions, pathological fractures, chronic bone pain, bone crisis, bone infarcts, osteonecrosis and skeletal deformities) are typical manifestations of the most prevalent form of the disease, the so-called non-neuronopathic type 1. However, severity and coexistence of different symptoms are highly variable. The determination of deficient β-glucocerebrosidase activity in leukocytes or fibroblasts by enzymatic assay is the gold standard for the diagnosis of Gaucher disease. Comprehensive and reproducible evaluation and monitoring of all clinically relevant aspects are fundamental for the effective management of Gaucher disease patients. Enzyme replacement therapy has been shown to be effective in reducing glucocerebroside storage burden and diminishing the deleterious effects caused by its accumulation. Tailored treatment plan for each patient should be directed to symptom relief, general improvement of quality of life, and prevention of irreversible damage. PMID:26604942

  14. Pituitary tumors: pathophysiology, clinical manifestations and management.

    PubMed

    Arafah, B M; Nasrallah, M P

    2001-12-01

    Pituitary tumors are frequently encountered intracranial neoplasms. They present with a variety of clinical manifestations that include symptoms and signs of excessive hormone secretion by the tumor, signs of hormone deficits by the normal pituitary gland and others related to expansion of the tumor mass and the resulting compression of surrounding structures such as the optic chiasm and cranial nerves. Advances in molecular biology, immunocytochemical staining and imaging, and the introduction of new treatment options have improved our understanding of the natural history of these adenomas and their management. Available treatments include surgical, medical and radiation therapy. Although the primary treatment for each tumor type may vary, it is important to consider all available options and select the most applicable for that patient. The interaction of all members of management team, including the primary care provider, the endocrinologist and the neurosurgeon in selecting the treatment course can only improve therapeutic outcome. Regardless of the initial choice of treatment,follow-up of all patients should be maintained indefinitely. The managing physician should be familiar with the natural history and long-term complications of pituitary adenomas, and with the side effects of treatments given over the years. PMID:11733226

  15. Emotional manifestations of PD: Neurobiological basis.

    PubMed

    Castrioto, Anna; Thobois, Stéphane; Carnicella, Sebastien; Maillet, Audrey; Krack, Paul

    2016-08-01

    Neuropsychiatric symptoms are common and disabling in PD. Their neurobiological bases are complex, partly because of the disease itself and partly because of the dopaminergic treatment. The aim of this review is to focus on the emotional manifestations stemming from the neurodegenerative process itself. We focus on depression, anxiety, apathy, and fatigue, which can all be part of the clinical spectrum of premotor disease and may be improved or masked by medications targeting parkinsonian motor signs or psychiatric symptoms as the disease progresses. Findings from clinical, neuroimaging, and animal studies are reviewed, showing a major contribution of the dopaminergic system to the pathophysiology of these disabling symptoms. Degeneration of noradrenergic and serotonergic projection systems also has an impact on psychiatric symptoms of PD. The available literature is reviewed, but at present there is a lack of studies that would allow disentangling the separate contribution of each of the monoaminergic systems. The use of a pragmatic classification of all these symptoms under the umbrella of hypodopaminergic behavioral syndrome seems clinically useful, as it emphasizes the crucial, although not exclusive, nature of their dopaminergic neurobiological basis, which has important implications in the clinical management of PD. © 2016 International Parkinson and Movement Disorder Society.

  16. ECG manifestations of left ventricular electrical remodeling.

    PubMed

    Estes, E Harvey

    2012-01-01

    Research and thinking about the electrocardiographic manifestations of left ventricular hypertrophy has been constrained by a limited conceptual model of the process: heart disease produces chamber enlargement (increased mass), which in turn produces an altered electrocardiogram. The process is much more complex than can be represented in this simple model. A more robust and intricate model is proposed, in which heart (and vascular) disease causes structural changes, electrical changes, biochemical changes, and others, all of which interact to produce electrical remodeling of ventricular myocardium. This electrical remodeling results in a variety of ECG changes. All of these changes interact, leading to an altered clinical course, and to premature death. It is suggested that research, based on this model, can provide new clues to the processes involved, and improve the prediction of clinical outcomes. New directions in research, in recording equipment, and in organizational activities are suggested to test this new model, and to improve the usefulness of the electrocardiogram as a research and diagnostic tool.

  17. Emotional manifestations of PD: Neurobiological basis.

    PubMed

    Castrioto, Anna; Thobois, Stéphane; Carnicella, Sebastien; Maillet, Audrey; Krack, Paul

    2016-08-01

    Neuropsychiatric symptoms are common and disabling in PD. Their neurobiological bases are complex, partly because of the disease itself and partly because of the dopaminergic treatment. The aim of this review is to focus on the emotional manifestations stemming from the neurodegenerative process itself. We focus on depression, anxiety, apathy, and fatigue, which can all be part of the clinical spectrum of premotor disease and may be improved or masked by medications targeting parkinsonian motor signs or psychiatric symptoms as the disease progresses. Findings from clinical, neuroimaging, and animal studies are reviewed, showing a major contribution of the dopaminergic system to the pathophysiology of these disabling symptoms. Degeneration of noradrenergic and serotonergic projection systems also has an impact on psychiatric symptoms of PD. The available literature is reviewed, but at present there is a lack of studies that would allow disentangling the separate contribution of each of the monoaminergic systems. The use of a pragmatic classification of all these symptoms under the umbrella of hypodopaminergic behavioral syndrome seems clinically useful, as it emphasizes the crucial, although not exclusive, nature of their dopaminergic neurobiological basis, which has important implications in the clinical management of PD. © 2016 International Parkinson and Movement Disorder Society. PMID:27041545

  18. Monogenic Autoinflammatory Diseases: Concept And Clinical Manifestations

    PubMed Central

    De Jesus, Adriana Almeida; Goldbach-Mansky, Raphaela

    2013-01-01

    The objectives of this review are to describe the clinical manifestations of the growing spectrum of monogenic autoinflammatory diseases including recently described syndromes. The autoinflammatory diseases can be grouped based on clinical findings: 1. the three classic hereditary “periodic fever syndromes”, familial Mediterranean Fever (FMF); TNF receptor associated periodic syndrome (TRAPS); and mevalonate kinase deficiency/hyperimmunoglobulinemia D and periodic fever syndrome (HIDS); 2. the cryopyrin associated periodic syndromes (CAPS), comprising familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID) or CINCA, and; 3. pediatric granulomatous arthritis (PGA); 4. disorders presenting with skin pustules, including deficiency of interleukin 1 receptor antagonist (DIRA); Majeed syndrome; pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome; deficiency of interleukin 36 receptor antagonist (DITRA); CARD14 mediated psoriasis (CAMPS), and early-onset inflammatory bowel diseases (EO-IBD); 5. inflammatory disorders caused by mutations in proteasome components, the proteasome associated autoinflammatory syndromes (PRAAS) 6. very rare conditions presenting with autoinflammation and immunodeficiency. PMID:23711932

  19. Oral manifestations of lymphoma: a systematic review

    PubMed Central

    Silva, Taísa Domingues Bernardes; Ferreira, Camila Belo Tavares; Leite, Gustavo Boehmer; de Menezes Pontes, José Roberto; Antunes, Héliton S

    2016-01-01

    Lymphoma is a malignant disease with two forms: Hodgkin’s lymphoma (HL) and non-Hodgkin’s lymphoma (NHL). Non-Hodgkin’s lymphoma is diagnosed in extranodal sites in 40% of cases, and the head and neck region is the second most affected, with an incidence of 11–33%, while HL has a very low incidence in extranodal sites (1–4%). The aim of this study was to identify the oral manifestations of lymphoma through a systematic literature review, which we conducted using the PubMed, Lilacs, Embase, and Cochrane Library databases. We found 1456 articles, from which we selected 73. Among the intraoral findings, the most frequent were ulcerations, pain, swelling, and tooth mobility, while the extraoral findings included facial asymmetry and cervical, submandibular, and submental lymphadenopathy. Among the few studies reporting imaging findings, the most cited lesions included hypodense lesions with diffuse boundaries, bone resorptions, and tooth displacements. The publications reviewed highlight gaps in the areas of early detection, diagnosis, and proper treatment. PMID:27594910

  20. Ophthalmic Manifestations of Sickle Cell Disease.

    PubMed

    Scott, Adrienne W

    2016-09-01

    Sickle cell disease (SCD), the most common inherited blood disorder, is characterized by defective oxygen transport. Every part of the eye can be affected by microvascular occlusions from SCD; however, the major cause of vision loss is proliferative sickle cell retinopathy (PSR). Although individuals with the HbSS genotype of SCD manifest more systemic morbidity and those with the HbSC genotype have a milder clinical course, those with HbSC have an increased risk of developing PSR and resultant vision loss. Sickle cell retinopathy has a variable phenotype, even among individuals with the same genotype. Most patients with SCD maintain good vision because the associated retinopathy occurs in the retinal periphery, and any associated "sea fan" neovascularization has a high tendency to autoinfarct and regress. Vision loss from PSR is largely preventable via regular retinal examinations and treatment as indicated. Novel retinal imaging techniques such as wide-field fluorescein angiography, spectral domain optical coherence tomography, and optical coherence tomography angiography can identify evidence of retinal microvascular occlusions in most patients with SCD. Further study is necessary to discover which individuals are at highest risk for vision loss, which of these retinal imaging modalities is clinically important, and which systemic treatments may decrease risk of vision loss from sickle cell retinopathy. PMID:27598358

  1. Radiation nephritis. Clinical manifestations and pathophysiologic mechanisms

    SciTech Connect

    Krochak, R.J.; Baker, D.G.

    1986-05-01

    Radiation nephritis is both volume and dose related. Clinical experience would indicate that a minimum of one third of the renal volume needs to be excluded from nephrotoxic doses which appears to have a threshold of 2,000 cGy. The site of damage leading to renal failure appears to be the microvasculature ultimately expressed as glomerulosclerosis. How much direct damage to the tubular system contributes to this process is unclear, but undoubtedly the resultant systemic physiologic effects potentiate the expression of damage in the irradiated kidney. The acute syndrome, with all the potential manifestations of renal failure, rarely presents sooner than six months and appears to have no clear prodrome, although it would seem reasonable that a subclinical syndrome consisting of abnormalities detectable by urinalysis may occur. Treatment of radiation-induced nephritis or hypertension is no different from treatment for nephritis from any other cause and should be aggressive with lifelong follow-up. Carcinogenesis is a rare late expression of radiation-induced kidney damage. 25 references.

  2. Oral manifestations of lymphoma: a systematic review

    PubMed Central

    Silva, Taísa Domingues Bernardes; Ferreira, Camila Belo Tavares; Leite, Gustavo Boehmer; de Menezes Pontes, José Roberto; Antunes, Héliton S

    2016-01-01

    Lymphoma is a malignant disease with two forms: Hodgkin’s lymphoma (HL) and non-Hodgkin’s lymphoma (NHL). Non-Hodgkin’s lymphoma is diagnosed in extranodal sites in 40% of cases, and the head and neck region is the second most affected, with an incidence of 11–33%, while HL has a very low incidence in extranodal sites (1–4%). The aim of this study was to identify the oral manifestations of lymphoma through a systematic literature review, which we conducted using the PubMed, Lilacs, Embase, and Cochrane Library databases. We found 1456 articles, from which we selected 73. Among the intraoral findings, the most frequent were ulcerations, pain, swelling, and tooth mobility, while the extraoral findings included facial asymmetry and cervical, submandibular, and submental lymphadenopathy. Among the few studies reporting imaging findings, the most cited lesions included hypodense lesions with diffuse boundaries, bone resorptions, and tooth displacements. The publications reviewed highlight gaps in the areas of early detection, diagnosis, and proper treatment.

  3. Rheumatic manifestations in inflammatory bowel disease.

    PubMed

    Atzeni, Fabiola; Defendenti, Caterina; Ditto, Maria Chiara; Batticciotto, Alberto; Ventura, Donatella; Antivalle, Marco; Ardizzone, Sandro; Sarzi-Puttini, Piercarlo

    2014-01-01

    Musculoskeletal symptoms (articular, periarticular and muscular involvement, osteoporosis and related fractures, and fibromyalgia) are the most common frequent extra-intestinal manifestations of inflammatory bowel disease (IBD) and affect 6-46% of patients. IBD-related arthropathy is one of a group of inflammatory arthritides known as seronegative spondyloarthropathies (SpA), which also includes idiopathic ankylosing spondylitis (AS), reactive arthritis, psoriatic arthritis, and undifferentiated SpA. The articular involvement in IBD significantly affects the patients' quality of the life. Although magnetic resonance imaging (MRI) is still the gold standard for assessing entheseal involvement, ultrasonography (US) is a non-invasive and easily reproducible means of detecting early pathological changes in SpA patients. It can identify characteristic features of SpA such as enthesitis, bone erosions, synovitis, bursitis, and tenosynovitis and is therefore helpful for diagnostic purposes. Anti-TNF drugs should be used to treat AS patients with axial and peripheral symptoms (arthritis and enthesitis) who have persistently high levels of disease activity despite conventional treatment, and adalimumab and infliximab can also be beneficially used in patients with IBD.

  4. Oral Manifestation in Mentally Challenged Children

    PubMed Central

    Rahul, V K; Mathew, Cinil; Jose, Sunil; Thomas, George; Noushad, M C; Feroz, T P Mohammed

    2015-01-01

    Background: In general, mentally challenged children have higher rates poor oral hygiene, gingivitis and periodontitis than the general population. An investigation was undertaken to assess the oral manifestations of mentally challenged children in Chennai, India. Materials and Methods: The study group consisted of 150 children (70 Down syndrome patients and 80 cerebral palsy patients). Of which, 93 patients were males and 57 were females. Results: Speech difficulty hindered the communication between the patient and the dentist. Mastication and swallowing difficulties were also present in few children. Profuse salivation was a cause for drooling of saliva down the cheeks, which was a constant finding in cerebral palsy children. The oral hygiene statuses of the patient were significantly poor. The prevalence of periodontitis was 35.7% in Down syndrome and 55.0% in cerebral palsy patients. Whereas, the prevalence of gingivitis was found to be 92.9% and 61.3% respectively. The prevalence of fractured maxillary anterior teeth was found to be more evident in cerebral palsy patients (62.9%) when compared to Down syndrome patients (0.0%). An increase in age shows an increase in the decayed-missing-filled teeth which is statistically significant. Conclusion: The prominent findings like flat nasal bridge (94.3%), hypertelorism (92.9%), high arched palate (78.6%) and fissured tongue (78.6%) in our study, suggest that they could be used as a reliable clinical markers to diagnose Down syndrome condition. PMID:25859105

  5. Oral manifestations of hepatitis C virus infection

    PubMed Central

    Carrozzo, Marco; Scally, Kara

    2014-01-01

    Extrahepatic manifestations (EHMs) of hepatitis C virus (HCV) infection can affect a variety of organ systems with significant morbidity and mortality. Some of the most frequently reported EHM of HCV infection, involve the oral region predominantly or exclusively. Oral lichen planus (OLP) is a chronic inflammatory condition that is potentially malignant and represents cell-mediated reaction to a variety of extrinsic antigens, altered self-antigens, or super antigens. Robust epidemiological evidence support the link between OLP and HCV. As the virus may replicate in the oral mucosa and attract HCV-specific T lymphocytes, HCV may be implicated in OLP pathogenesis. Sjögren syndrome (SjS) is an autoimmune exocrinopathy, characterized by dryness of the mouth and eyes and a multitude of other systemic signs and symptoms. SjS patients have also an increased risk of non-Hodgkin lymphoma. Patients with chronic hepatitis C do frequently have histological signs of Sjögren-like sialadenitis with mild or even absent clinical symptoms. However, it is still unclear if HCV may cause a disease mimicking SjS or it is directly responsible for the development of SjS in a specific subset of patients. Oral squamous cell carcinoma is the most common oral malignant tumour and at least in some part of the world could be linked to HCV. PMID:24976694

  6. Oral manifestations of hepatitis C virus infection.

    PubMed

    Carrozzo, Marco; Scally, Kara

    2014-06-28

    Extrahepatic manifestations (EHMs) of hepatitis C virus (HCV) infection can affect a variety of organ systems with significant morbidity and mortality. Some of the most frequently reported EHM of HCV infection, involve the oral region predominantly or exclusively. Oral lichen planus (OLP) is a chronic inflammatory condition that is potentially malignant and represents cell-mediated reaction to a variety of extrinsic antigens, altered self-antigens, or super antigens. Robust epidemiological evidence support the link between OLP and HCV. As the virus may replicate in the oral mucosa and attract HCV-specific T lymphocytes, HCV may be implicated in OLP pathogenesis. Sjögren syndrome (SjS) is an autoimmune exocrinopathy, characterized by dryness of the mouth and eyes and a multitude of other systemic signs and symptoms. SjS patients have also an increased risk of non-Hodgkin lymphoma. Patients with chronic hepatitis C do frequently have histological signs of Sjögren-like sialadenitis with mild or even absent clinical symptoms. However, it is still unclear if HCV may cause a disease mimicking SjS or it is directly responsible for the development of SjS in a specific subset of patients. Oral squamous cell carcinoma is the most common oral malignant tumour and at least in some part of the world could be linked to HCV. PMID:24976694

  7. Oral manifestations of lymphoma: a systematic review.

    PubMed

    Silva, Taísa Domingues Bernardes; Ferreira, Camila Belo Tavares; Leite, Gustavo Boehmer; de Menezes Pontes, José Roberto; Antunes, Héliton S

    2016-01-01

    Lymphoma is a malignant disease with two forms: Hodgkin's lymphoma (HL) and non-Hodgkin's lymphoma (NHL). Non-Hodgkin's lymphoma is diagnosed in extranodal sites in 40% of cases, and the head and neck region is the second most affected, with an incidence of 11-33%, while HL has a very low incidence in extranodal sites (1-4%). The aim of this study was to identify the oral manifestations of lymphoma through a systematic literature review, which we conducted using the PubMed, Lilacs, Embase, and Cochrane Library databases. We found 1456 articles, from which we selected 73. Among the intraoral findings, the most frequent were ulcerations, pain, swelling, and tooth mobility, while the extraoral findings included facial asymmetry and cervical, submandibular, and submental lymphadenopathy. Among the few studies reporting imaging findings, the most cited lesions included hypodense lesions with diffuse boundaries, bone resorptions, and tooth displacements. The publications reviewed highlight gaps in the areas of early detection, diagnosis, and proper treatment. PMID:27594910

  8. [Tumorous extragenital manifestation of lymphogranuloma venereum].

    PubMed

    Trebing, Dietrich; Brunner, Martina; Kröning, Yvonne; Seele, Peggy

    2005-06-01

    A 36-year-old homosexual man presented with a 3-week history of pin-head-sized vesicles on the lower lip followed by a tumorous infiltrate, cervical lymphadenopathy and episodic fevers. Laboratory findings included striking leukocytosis and elevated inflammatory markers. Serologically,anti-chlamydial antibodies (IgG, IgA,IgM) were present. The histological findings were compatible with a chlamydial infection; PCR examination of the tumor and lymph nodes was positive for Chlamydia trachomatis DNA. Treatment with doxycycline (2x 100 mg/daily p.o. for 3 weeks) led to rapid improvement. Lymphogranuloma venereum is a relatively rare, worldwide illness, more common in tropical and sub-tropical zones. In Germany, there are 10-50 cases reported annually but many cases go unreported. The causative organism is Chlamydia trachomatis, serotypes L1-3. The transfer occurs through skin or mucous membrane contact with an infected partner. In the advanced stage, the infection can lead to marked internal organ involvement. The special features of our case include the extragenital manifestation and the tumorous appearance of the illness which is very rarely diagnosed in Germany. PMID:15892848

  9. Behavioural manifestations of anabolic steroid use.

    PubMed

    Trenton, Adam J; Currier, Glenn W

    2005-01-01

    The use of anabolic androgenic steroids (AAS) for gains in strength and muscle mass is relatively common among certain subpopulations, including athletes, bodybuilders, adolescents and young adults. Adverse physical effects associated with steroid abuse are well documented, but more recently, increased attention has been given to the adverse psychiatric effects of these compounds. Steroids may be used in oral, 17alpha-alkylated, or intramuscular, 17beta-esterified, preparations. Commonly, steroid users employ these agents at levels 10- to 100-fold in excess of therapeutic doses and use multiple steroids simultaneously, a practice known as 'stacking'. Significant psychiatric symptoms including aggression and violence, mania, and less frequently psychosis and suicide have been associated with steroid abuse. Long-term steroid abusers may develop symptoms of dependence and withdrawal on discontinuation of AAS. Treatment of AAS abusers should address both acute physical and behavioural symptoms as well as long-term abstinence and recovery. To date, limited information is available regarding specific pharmacological treatments for individuals recovering from steroid abuse. This paper reviews the published literature concerning the recognition and treatment of behavioural manifestations of AAS abuse.

  10. Sickle cell disease: renal manifestations and mechanisms

    PubMed Central

    Nath, Karl A.; Hebbel, Robert P.

    2015-01-01

    Sickle cell disease (SCD) substantially alters renal structure and function, and causes various renal syndromes and diseases. Such diverse renal outcomes reflect the uniquely complex vascular pathobiology of SCD and the propensity of red blood cells to sickle in the renal medulla because of its hypoxic, acidotic, and hyperosmolar conditions. Renal complications and involvement in sickle cell nephropathy (SCN) include altered haemodynamics, hypertrophy, assorted glomerulopathies, chronic kidney disease, acute kidney injury, impaired urinary concentrating ability, distal nephron dysfunction, haematuria, and increased risks of urinary tract infections and renal medullary carcinoma. SCN largely reflects an underlying vasculopathy characterized by cortical hyperperfusion, medullary hypoperfusion, and an increased, stress-induced vasoconstrictive response. Renal involvement is usually more severe in homozygous disease (sickle cell anaemia, HbSS) than in compound heterozygous types of SCD (for example HbSC and HbSβ+-thalassaemia), and is typically mild, albeit prevalent, in the heterozygous state (sickle cell trait, HbAS). Renal involvement contributes substantially to the diminished life expectancy of patients with SCD, accounting for 16–18% of mortality. As improved clinical care promotes survival into adulthood, SCN imposes a growing burden on both individual health and health system costs. This Review addresses the renal manifestations of SCD and focuses on their underlying mechanisms. PMID:25668001

  11. Different manifestations of calcifying cystic odontogenic tumor.

    PubMed

    Utumi, Estevam Rubens; Pedron, Irineu Gregnanin; da Silva, Leopoldo Penteado Nucci; Machado, Gustavo Grothe; Rocha, André Caroli

    2012-01-01

    The calcifying cystic odontogenic tumor normally presents as a painless, slow-growing mass, involving both maxilla and mandible, primarily the anterior segment (incisor/canine area). It generally affects young adults in the third to fourth decades, with no gender predilection. Computerized tomography images revealed important characteristics that were not detected by panoramic radiography, such as fenestration, calcification and tooth-like structures. The typical microscopic feature of this lesion is the presence of variable amounts of aberrant epithelial cells, without nuclei, which are named "ghost cells". In addition, dysplastic dentine can be found and occasionally the cyst can be associated with an area of dental hard tissue formation resembling an odontoma. The treatment for calcifying cystic odontogenic tumor involves simple enucleation and curettage. The purpose of this article is to present two different manifestation of calcifying cystic odontogenic tumor in which computerized tomography, associated to clinical features, served as an important tool for diagnosis, adequate surgical planning and follow-up of patients.

  12. Detection of disease outbreaks by the use of oral manifestations.

    PubMed

    Torres-Urquidy, M H; Wallstrom, G; Schleyer, T K L

    2009-01-01

    Oral manifestations of diseases caused by bioterrorist agents could be a potential data source for biosurveillance. This study had the objectives of determining the oral manifestations of diseases caused by bioterrorist agents, measuring the prevalence of these manifestations in emergency department reports, and constructing and evaluating a detection algorithm based on them. We developed a software application to detect oral manifestations in free text and identified positive reports over three years of data. The normal frequency in reports for oral manifestations related to anthrax (including buccal ulcers-sore throat) was 7.46%. The frequency for tularemia was 6.91%. For botulism and smallpox, the frequencies were 0.55% and 0.23%. We simulated outbreaks for these bioterrorism diseases and evaluated the performance of our system. The detection algorithm performed better for smallpox and botulism than for anthrax and tularemia. We found that oral manifestations can be a valuable tool for biosurveillance. PMID:19131324

  13. Onset Manifestations of Spinal and Bulbar Muscular Atrophy (Kennedy's Disease).

    PubMed

    Finsterer, Josef; Soraru, Gianni

    2016-03-01

    Spinal and bulbar muscular atrophy (SBMA) is regarded as a disorder with adult onset between third and fifth decade of life. However, there is increasing evidence that SBMA may start already before adulthood. The present study investigated the following: (1) Which clinical manifestations have been described so far in the literature as initial manifestations? (2) Which was the age at onset of these manifestations? and (3) Is age at onset dependent on the CAG-repeat length if non-motor manifestations are additionally considered? Data for this review were identified by searches of MEDLINE using appropriate search terms. Onset manifestations in SBMA can be classified as frequent, rare, motor, non-motor, or questionable. Frequent are muscle weakness, cramps, fasciculations/twitching, tremor, dysarthria, dysphagia, or gynecomastia. Rare are myalgia, easy fatigability, exercise intolerance, polyneuropathy, hyper-CKemia, under-masculinized genitalia, scrotal hypospadias, microphallus, laryngospasm, or oligospermia. Questionable manifestations include sensory disturbances, cognitive impairment, increased pituitary volume, diabetes, reduced tongue pressure, elevated creatine-kinase, or low androgens/high estrogens. Age at onset is highly variable ranging from 4-76 years. Non-motor manifestations develop usually before motor manifestations. Age at onset depends on what is considered as an onset manifestation. Considering non-motor onset manifestations, age at onset is independent of the CAG-repeat size. In conclusion, age at onset of SBMA depends on what is regarded as onset manifestation. If non-motor manifestations are additionally considered, age at onset is independent of the CAG-repeat length. Since life expectancy is hardly reduced in SBMA, re-investigation of patients from published studies with regard to their initial disease profiles is recommended. PMID:26482145

  14. Acute myeloid leukemia with non-specific cutaneous manifestation.

    PubMed

    Kotokey, R K; Potsangham, T; Das, R

    2008-09-01

    Acute myeloid leukemia is not uncommon in upper Assam. Primary skin manifestation in AML though very rare, may be found. The skin manifestation may be the first presentation in AML. Here such a case has been discussed which presented with primarily skin manifestation, subsequently diagnosed as AML. Therefore routine investigations are mandatory in all patients before going for a sophisticated investigation so that the diagnosis is not missed. PMID:19086364

  15. Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (II): thrombocytopenia and skin manifestations.

    PubMed

    Cervera, R; Tektonidou, M G; Espinosa, G; Cabral, A R; González, E B; Erkan, D; Vadya, S; Adrogué, H E; Solomon, M; Zandman-Goddard, G; Shoenfeld, Y

    2011-02-01

    The objectives of the 'Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations' were to assess the clinical utility of the international consensus statement on classification criteria and treatment guidelines for the catastrophic APS, to identify and grade the studies that analyze the relationship between the antiphospholipid antibodies and the non-criteria APS manifestations, and to present the current evidence regarding the accuracy of these non-criteria APS manifestations for the detection of patients with APS. This article summarizes the studies analyzed on thrombocytopenia and skin manifestations, and presents the recommendations elaborated by the Task Force after this analysis.

  16. 19 CFR 122.74 - Incomplete (pro forma) manifest.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 19 Customs Duties 1 2011-04-01 2011-04-01 false Incomplete (pro forma) manifest. 122.74 Section 122.74 Customs Duties U.S. CUSTOMS AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT... Aircraft Departing From the United States § 122.74 Incomplete (pro forma) manifest. (a)...

  17. 19 CFR 122.74 - Incomplete (pro forma) manifest.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 19 Customs Duties 1 2010-04-01 2010-04-01 false Incomplete (pro forma) manifest. 122.74 Section 122.74 Customs Duties U.S. CUSTOMS AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY; DEPARTMENT... Aircraft Departing From the United States § 122.74 Incomplete (pro forma) manifest. (a)...

  18. 40 CFR 761.209 - Retention of manifest records.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... Section 761.209 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.209 Retention of manifest records. (a) A generator of PCB waste shall keep a copy of each manifest signed in accordance with § 761.208(a)(1) until...

  19. 40 CFR 761.208 - Use of the manifest.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 761.208 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.208 Use of the manifest. (a)(1) The generator of PCB waste shall: (i) Sign the manifest certification by hand. (ii) Obtain the handwritten signature of...

  20. 40 CFR 761.209 - Retention of manifest records.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... Section 761.209 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.209 Retention of manifest records. (a) A generator of PCB waste shall keep a copy of each manifest signed in accordance with § 761.208(a)(1) until...

  1. 40 CFR 761.208 - Use of the manifest.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 761.208 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.208 Use of the manifest. (a)(1) The generator of PCB waste shall: (i) Sign the manifest certification by hand. (ii) Obtain the handwritten signature of...

  2. 40 CFR 761.209 - Retention of manifest records.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... Section 761.209 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.209 Retention of manifest records. (a) A generator of PCB waste shall keep a copy of each manifest signed in accordance with § 761.208(a)(1) until...

  3. 40 CFR 761.208 - Use of the manifest.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 761.208 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES CONTROL... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.208 Use of the manifest. (a)(1) The generator of PCB waste shall: (i) Sign the manifest certification by hand. (ii) Obtain the handwritten signature of...

  4. A Review of the Latent and Manifest Benefits (LAMB) Scale

    ERIC Educational Resources Information Center

    Muller, Juanita; Waters, Lea

    2012-01-01

    The latent and manifest benefits (LAMB) scale (Muller, Creed, Waters & Machin, 2005) was designed to measure the latent and manifest benefits of employment and provide a single scale to test Jahoda's (1981) and Fryer's (1986) theories of unemployment. Since its publication in 2005 there have been 13 studies that have used the scale with 5692…

  5. 40 CFR 263.25 - Electronic manifest signatures.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 40 Protection of Environment 26 2014-07-01 2014-07-01 false Electronic manifest signatures. 263.25 Section 263.25 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS APPLICABLE TO TRANSPORTERS OF HAZARDOUS WASTE Compliance With the Manifest System...

  6. 40 CFR 264.71 - Use of manifest system.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... electronic signature within the meaning of 40 CFR 262.25. (2) Any requirement in these regulations to give... for a manifest to accompany a hazardous waste shipment is satisfied when a copy of an electronic... date of delivery of the waste. (j) Imposition of user fee for electronic manifest use. An owner...

  7. 76 FR 36363 - Hazardous Waste Manifest Printing Specifications Correction Rule

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-06-22

    ... standardized manifest form during the promulgation of the March 4, 2005 Manifest Form Revisions Rule (70 FR... Executive Order 12866 (58 FR 51735, October 4, 1993), the Agency must determine whether the regulatory... and is therefore not subject to review under Executive Orders 12866 and 13563 (76 FR 3821, January...

  8. 40 CFR 262.25 - Electronic manifest signatures.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 40 Protection of Environment 26 2014-07-01 2014-07-01 false Electronic manifest signatures. 262.25 Section 262.25 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS APPLICABLE TO GENERATORS OF HAZARDOUS WASTE The Manifest § 262.25 Electronic...

  9. 46 CFR 148.02-3 - Dangerous cargo manifest.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 46 Shipping 5 2010-10-01 2010-10-01 false Dangerous cargo manifest. 148.02-3 Section 148.02-3 Shipping COAST GUARD, DEPARTMENT OF HOMELAND SECURITY (CONTINUED) DANGEROUS CARGOES CARRIAGE OF SOLID HAZARDOUS MATERIALS IN BULK Vessel Requirements § 148.02-3 Dangerous cargo manifest. (a) Each vessel,...

  10. Reversion of disease manifestations after HCV eradication.

    PubMed

    van der Meer, Adriaan J; Berenguer, Marina

    2016-10-01

    Chronic infection with the hepatitis C virus (HCV) may lead to hepatic fibrosis and eventually cirrhosis, at which stage, patients have a substantial risk of liver failure, hepatocellular carcinoma (HCC) and liver-related death. Moreover, HCV infection is associated with several extrahepatic manifestations which impact the quality of life and increase the non-liver-related mortality rate. For patients with compensated liver disease, interferon (IFN)-based antiviral therapy has been a treatment option for over two decades. Long-term follow-up studies indicated that among those with sustained virological response (SVR) the extend of hepatic fibrosis can regress and that their risk of cirrhosis-related complications (including HCC) is reduced, also in case of cirrhosis. Recent population-based studies extended these observations for solid extrahepatic outcomes, such as end-stage renal failure and cardiovascular events. Most importantly, SVR has been associated with prolonged overall survival. These results highlight the importance of the development of new direct-acting antivirals (DAAs), by which almost all patients are able to eradicate HCV in a comfortable manner. Based on the excellent first experiences with the DAAs, physicians gained confidence to use these drugs among patients with decompensated cirrhosis on a more regular basis as well. This was not possible with interferon therapy. Also in this high risk population the DAAs show high SVR rates with improvements in biochemical parameters of liver function shortly after therapy, especially in case of SVR. In fact, some patients could actually be removed from the liver transplantation waiting list due to clinical improvement following DAA therapy. How these short-term results translate into a prolonged (long-term) survival has yet to be determined, as well as which patients with decompensated liver disease are likely or not to benefit from viral eradication. Here we review the current data regarding the

  11. Clinical and Genetic Characterization of Manifesting Carriers of DMD Mutations

    PubMed Central

    Soltanzadeh, Payam; Friez, Michael J.; Dunn, Diane; von Niederhausern, Andrew; Gurvich, Olga L.; Swoboda, Kathryn J.; Sampson, Jacinda B.; Pestronk, Alan; Connolly, Anne M.; Florence, Julaine M.; Finkel, Richard S.; Bönnemann, Carsten G.; Medne, Livija; Mendell, Jerry R.; Mathews, Katherine D.; Wong, Brenda L.; Sussman, Michael D.; Zonana, Jonathan; Kovak, Karen; Gospe, Sidney M.; Gappmaier, Eduard; Taylor, Laura E.; Howard, Michael T.; Weiss, Robert B.; Flanigan, Kevin M.

    2010-01-01

    Manifesting carriers of DMD gene mutations may present diagnostic challenges, particularly in the absence of a family history of dystrophinopathy. We review the clinical and genetic features in fifteen manifesting carriers identified among 860 subjects within the United Dystrophinopathy Project, a large clinical dystrophinopathy cohort whose members undergo comprehensive DMD mutation analysis. We defined manifesting carriers as females with significant weakness, excluding those with only myalgias/cramps. DNA extracted from peripheral blood was used to study X chromosome inactivation patterns. Among these manifesting carriers, age at symptom onset ranged from 2 to 47 years. Seven had no family history and eight had male relatives with Duchene muscular dystrophy (DMD). Clinical severity among the manifesting carriers varied from a DMD-like progression to a very mild Becker muscular dystrophy-like phenotype. Eight had exonic deletions or duplications and six had point mutations. One patient had two mutations (an exonic deletion and a splice site mutation), consistent with a heterozygous compound state. The X chromosome inactivation pattern was skewed toward nonrandom in four out of seven informative deletions or duplications but was random in all cases with nonsense mutations. We present the results of DMD mutation analysis in this manifesting carrier cohort, including the first example of a presumably compound heterozygous DMD mutation. Our results demonstrate that improved molecular diagnostic methods facilitate the identification of DMD mutations in manifesting carriers, and confirm the heterogeneity of mutational mechanisms as well as the wide spectrum of phenotypes. PMID:20630757

  12. Diagnosis and treatment of HIV-associated manifestations in otolaryngology

    PubMed Central

    lacovou, Emily; Vlastarakos, Petros V.; Papacharalampous, George; Kampessis, George; Nikolopoulos, Thomas P.

    2012-01-01

    Almost 30 years after its first description, HIV still remains a global pandemic. The present paper aims to review the current knowledge on the ear, nose and throat (ENT) manifestations of HIV infection, and present the available diagnostic and treatment options. A literature review was conducted in Medline and other available database sources. Information from related books was also included in the data analysis. It is well acknowledged that up to 80% of HIV-infected patients eventually develop ENT manifestations; among which, oral disease appears to be the most common. Oro-pharyngeal manifestations include candidiasis, periodontal and gingival disease, HSV and HPV infection, oral hairy leucoplakia, Kaposi's sarcoma, and non-Hodgkin's lymphoma. ENT manifestations in the neck can present as cervical lymphadenopathy or parotid gland enlargement. Respective nasal manifestations include sinusitis (often due to atypical bacteria), and allergic rhinitis. Finally, otological manifestations include otitis (externa, or media), inner ear involvement (sensorineural hearing loss, disequilibrium), and facial nerve palsy (up to 100 times more frequently compared to the general population). Although ENT symptoms are not diagnostic of the disease, they might be suggestive of HIV infection, or related to its progression and the respective treatment failure. ENT doctors should be aware of the ENT manifestations associated with HIV disease, and the respective diagnosis and treatment. A multi-disciplinary approach may be required to provide the appropriate level of care to HIV patients. PMID:24470939

  13. Clinical Manifestations Associated with Neurocysticercosis: A Systematic Review

    PubMed Central

    Carabin, Hélène; Ndimubanzi, Patrick Cyaga; Budke, Christine M.; Nguyen, Hai; Qian, Yingjun; Cowan, Linda Demetry; Stoner, Julie Ann; Rainwater, Elizabeth; Dickey, Mary

    2011-01-01

    Background The clinical manifestations of neurocysticercosis (NCC) are poorly understood. This systematic review aims to estimate the frequencies of different manifestations, complications and disabilities associated with NCC. Methods A systematic search of the literature published from January 1, 1990, to June 1, 2008, in 24 different electronic databases and 8 languages was conducted. Meta-analyses were conducted when appropriate. Results A total of 1569 documents were identified, and 21 included in the analysis. Among patients seen in neurology clinics, seizures/epilepsy were the most common manifestations (78.8%, 95%CI: 65.1%–89.7%) followed by headaches (37.9%, 95%CI: 23.3%–53.7%), focal deficits (16.0%, 95%CI: 9.7%–23.6%) and signs of increased intracranial pressure (11.7%, 95%CI: 6.0%–18.9%). All other manifestations occurred in less than 10% of symptomatic NCC patients. Only four studies reported on the mortality rate of NCC. Conclusions NCC is a pleomorphic disease linked to a range of manifestations. Although definitions of manifestations were very rarely provided, and varied from study to study, the proportion of NCC cases with seizures/epilepsy and the proportion of headaches were consistent across studies. These estimates are only applicable to patients who are ill enough to seek care in neurology clinics and likely over estimate the frequency of manifestations among all NCC cases. PMID:21629722

  14. Hepatic manifestations of non-steroidal inflammatory bowel disease therapy

    PubMed Central

    Hirten, Robert; Sultan, Keith; Thomas, Ashby; Bernstein, David E

    2015-01-01

    Inflammatory bowel disease (IBD) is composed of Crohn’s disease and ulcerative colitis and is manifested by both bowel-related and extraintestinal manifestations. Recently the number of therapeutic options available to treat IBD has dramatically increased, with each new medication having its own mechanism of action and side effect profile. A complete understanding of the hepatotoxicity of these medications is important in order to distinguish these complications from the hepatic manifestations of IBD. This review seeks to evaluate the hepatobiliary complications of non-steroid based IBD medications and aide providers in the recognition and management of these side-effects. PMID:26644815

  15. [Splenic granulomas - rare extraintestinal manifestation of Crohn's disease].

    PubMed

    Schulz, C; von Arnim, U; Kuester, D; Fischbach, F; Malfertheiner, P

    2010-12-01

    Extraintestinal manifestations of inflammatory bowel disease occur frequently in parallel to the inflammation in the bowel. The activity of extraintestinal manifestations is often divergent to the activity of intestinal inflammation. We here present the case of a rare extraintestinal manifestation of Crohn's disease and report on a 52-year-old patient with known Crohn's disease and primary sclerosing cholangitis (PSC) in clinical remission. Multiple lesions of the spleen were observed in routine MR tomography. The histological examination of a specimen obtained by MR-guided fine needle biopsy showed non-caseating epitheloid cell granulomas. The splenic granulomas regressed completely and spontaneously without specific immunmodulatory therapy.

  16. The tricho-rhino-phalangeal syndrome: oral manifestations and management.

    PubMed

    Roberts, T; Chetty, M; Stephen, L; Fieggen, K; Beighton, P

    2014-05-01

    Tricho-rhino-phalangeal Syndrome (TRPS) is a rare inherited dysplasia affecting hair, nasal structure and fingers. A literature review indicated that since first described, three types of manifestations have been identified. A Table summarising the oral manifestations demonstrates the variety of presentations. A South African male child presented with the syndrome and was found to show premature eruption of permanent teeth, a finding that has not been previously reported. His oro-facial manifestations also included malaligned and unerupted crowded teeth, bulbosity of the nasal tip and an elongated philtrum and evidence of mild intellectual impairment. A protocol has been developed to guide the future management of these cases.

  17. Search for BFKL-evolution manifestations at high energies

    NASA Astrophysics Data System (ADS)

    Kim, Victor T.

    2015-11-01

    A brief overview of searches for BFKL-evolution manifestations at high energies is presented. Data comparison with the predictions of BFKL-evolution in the leading logarithmic (LL) and next-to-leading logarithmic (NLL) approximations are discussed.

  18. Management of Musculoskeletal Manifestations in Inflammatory Bowel Disease.

    PubMed

    Sheth, Tejas; Pitchumoni, C S; Das, Kiron M

    2015-01-01

    Musculoskeletal manifestations are the most common extraintestinal manifestations in inflammatory bowel diseases. Some appendicular manifestations are independent of gut inflammation and are treated with standard anti-inflammatory strategies. On the other hand, axial involvement is linked to gut inflammatory activity; hence, there is a considerable amount of treatment overlap. Biological therapies have revolutionized management of inflammatory bowel diseases as well as of associated articular manifestations. Newer mechanisms driving gut associated arthropathy have surfaced in the past decade and have enhanced our interests in novel treatment targets. Introduction of biosimilar molecules is expected in the US market in the near future and will provide an opportunity for considerable cost savings on healthcare. A multidisciplinary approach involving a gastroenterologist, rheumatologist, and physical therapist is ideal for these patients.

  19. 19 CFR 123.22 - In-transit manifest.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... CUSTOMS RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22 In-transit... cars or other overland carriers transiting Mexico a manifest on Customs Form 7512-B or 7533-C shall...

  20. Dust Acoustic Mode Manifestations in Earth's Dusty Ionosphere

    SciTech Connect

    Kopnin, S.I.; Popel, S.I.

    2005-10-31

    Dust acoustic mode manifestations in the dusty ionosphere are studied. The reason for an appearance of the low-frequency radio noises associated with such meteor fluxes as Perseids, Orionids, Leonids, and Gemenids is determined.

  1. [Oral and maxillofacial manifestations of familial adenomatosis polyposis. Gardner's syndrome].

    PubMed

    Wijn, M A; Keller, J J; Brand, H S

    2005-09-01

    Patients suffering from familial adenomatosis polyposis develop multiple pre-malignant gastrointestinal polyps and are at high risk of developing colon cancer. In addition extra-intestinal manifestations are observed frequently. The combination of extra-intestinal manifestations and familial adenomatosis polyposis is named Gardner's syndrome. An early diagnosis of this disease is important because it could mean a better prognosis for the patient. This review describes the oral and maxillofacial symptoms of FAP, and its potential implications for dental treatment. PMID:16184913

  2. Onychomadesis with Lichen Planus: An Under-Recognized Manifestation

    PubMed Central

    Grover, Chander; Vohra, Suruchi

    2015-01-01

    Onychomadesis or proximal separation of the nail pate usually results from severe, generalized dermatoses like bullous diseases, hand foot and mouth disease, varicella or severe cutaneous drug reactions. Although lichen planus (LP) produces varied nail manifestations (longitudinal onychorrhexis, onychoschizia, nail pigmentation, pterygium etc.), to the best of our knowledge, onychomadesis as a manifestation of nail LP is not recorded. This report presents two children with onychomadesis arising with generalized eruptive LP. PMID:26288428

  3. Mucocutaneous manifestations of acquired hypoparathyroidism: An observational study.

    PubMed

    Sarkar, Somenath; Mondal, Modhuchanda; Das, Kapildev; Shrimal, Arpit

    2012-09-01

    Hypoparathyroidism is a disorder of calcium and phosphorus metabolism due to decreased secretion of parathyroid hormone. Hypoparathyroidism can be hereditary and acquired. Acquired hypoparathyroidism usually occurs following neck surgery (thyroid surgery or parathyroid surgery). Along with systemic manifestations, hypoparathyroidism produces some skin manifestations. Lack of study regarding mucocutaneous manifestations of acquired hypoparathyroidism prompted us to undertake this study. To evaluate the mucocutaneous manifestations of acquired hypoparathyroidism. An observational study done in a tertiary care hospital of Kolkata by comprehensive history taking, through clinical examination and relevant laboratory investigations. Twenty-one patients were included in the study. The commonest form of acquired hypoparathyroidism was neck surgery (thyroidectomy and parathyroidectomy operation). Mucocutaneous manifestations were present in 76.19% of patients. The most frequent mucocutaneous manifestation was found in the hairs like the loss of axillary hair (61.9%), loss of pubic hair (52.38%), coarsening of body hair (47.62%), and alopecia areata (9.52%). The nail changes noted were brittle and ridged nail, followed by onycholysis, onychosezia, and onychomedesis. The most common skin features were xerotic skin in 11 patients (52.38%), followed by pellagra-like skin pigmentation, pustular psoriasis and acne form eruption, bullous impetigo, etc. Mucosa was normal in all the cases excepting the one which showed oral candidiasis. PMID:23087872

  4. Manifest dream content as a possible predictor of suicidality.

    PubMed

    Glucksman, Myron L

    2014-12-01

    The prediction of suicidal intent remains a clinical problem. This presentation illustrates that a distinction may be made between the manifest dream reports of patients who are potentially or acutely suicidal and those who are not. A review of the literature reveals that the manifest dream reports of clinically depressed, non-suicidal individuals differ from those who are depressed and acutely suicidal. The former contain themes of loss, disappointment, rejection, helplessness, hopelessness, failure, and death. The latter contain themes of dying, death, destruction, and violence directed toward the dreamer or others, as well as hopelessness and helplessness. The author collected manifest dream reports from three clinically depressed, non-suicidal patients and three clinically depressed, potentially or acutely suicidal patients. There are apparent differences between the themes of manifest dream reports in the clinically depressed, non-suicidal patients and the clinically depressed, potentially or acutely suicidal patients. The former contain themes of death, loss, rejection, vulnerability, hopelessness, and helplessness. The latter contain themes of active harm or violence (specifically toward the dreamer), dying or being dead, aloneness, vulnerability, hopelessness, and helplessness. Clinical cases and corresponding manifest dream reports are presented. Although this is a preliminary study, it is possible that manifest dream content may be used as one of the predictors of suicidality, in conjunction with latent dream content, diagnosis, life circumstance, and clinical status. PMID:25494585

  5. Musculoskeletal manifestations in diabetic patients at a tertiary center

    PubMed Central

    Attar, Suzan M.

    2012-01-01

    Objectives Diabetes mellitus is a major public health problem worldwide. Most diabetic patients will develop functional disabilities due to multiple factors, including musculoskeletal (MSK) manifestations. The purpose of this study was to determine the frequency of MSK in diabetic patients and to examine the possible predictors for its development. Methods We performed a cross-sectional study from June 1, 2010, to June 30, 2011, to evaluate MSK manifestations in adult diabetic patients at an outpatient clinic of King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Baseline variables were examined to determine predictors for the development of MSK complications. Analyses were carried out using the Statistical Package for Social sciences. Results We included 252 diabetic patients; 45 (17.9%) had MSK manifestations. Of these 45 patients, 41 (91.1%) had type 2 diabetes. The most common manifestations were carpal tunnel syndrome (n=17, 6.7%), shoulder adhesive capsulitis (n=17, 6.7%), and diabetic amyotrophy (n=12, 4.8%). A significant association was found between the development of MSK manifestations and manual labor, overweight, and vascular complications. On logistic regression analysis, the presence of vascular complications in general (B-coefficient=1.27, odds ratio=3.57, P<0.05, 95% confidence interval=1.31–9.78), and retinopathy in particular (B-coefficient=1.17, odds ratio=3.21, P<0.05, 95% confidence interval=1.47–7.02) can predict the development of MSK manifestations in about 82% of the cases. Conclusion Musculoskeletal manifestations are under recognized in adult diabetic patients, occurring in 18% of the cases. Physicians should consider examining the periarticular region of the joints in the hands and shoulders whenever a diabetic patient presents with MSK symptoms. PMID:23115579

  6. Holism and life manifestations: molecular and space-time biology.

    PubMed

    Krecek, J

    2010-01-01

    Appeals of philosophers to look for new concepts in sciences are being met with a weak response. Limited attention is paid to the relation between synthetic and analytic approach in solving problems of biology. An attempt is presented to open a discussion on a possible role of holism. The term "life manifestations" is used in accordance with phenomenology. Multicellular creatures maintain milieu intérieur to keep an aqueous milieu intracellulair in order to transform the energy of nutrients into the form utilizable for driving cellular life manifestations. Milieu intérieur enables to integrate this kind of manifestations into life manifestations of the whole multicellular creatures. The integration depends on a uniqueness and uniformity of the genome of cells, on their mutual recognition and adherence. The processes of ontogenetic development represent the natural mode of integration of cellular life manifestations. Functional systems of multicellular creatures are being established by organization of integrable cells using a wide range of developmental processes. Starting from the zygote division the new being displays all properties of a whole creature, although its life manifestations vary. Therefore, the whole organism is not only more than its parts, as supposed by holism, but also more than developmental stages of its life manifestations. Implicitly, the units of whole multicellular creature are rather molecular and developmental events than the cells per se. Holism, taking in mind the existence of molecular and space-time biology, could become a guide in looking for a new mode of the combination of analytical and synthetic reasoning in biology.

  7. Rheumatologic Manifestations in Iranian Patients with Autoimmune Thyroid Diseases

    PubMed Central

    Hezarkhani, Sharabeh; Aghaei, Mehrdad; Shamekhi, Maryam; Nomali, Mahin

    2014-01-01

    Background: Autoimmune thyroid diseases (ATDs) are the most common endocrine diseases which result in rheumatologic manifestations. Some studies have shown association between rheumatologic disorders and ATDs. Thus, the aim of this study was to assess the frequency of rheumatologic manifestations in patients with ATDs. Materials and Methods: In this cross-sectional descriptive study during 2010 to 2011, 65 patients with ATDs referred to the Rheumatology clinic of 5 Azar Hospital in Gorgan (North of Iran) were studied via systematic random sampling and patients with positive antithyroid peroxides (anti-TPO) were included in the study. These patients were examined by a rheumatologist for diagnosis of rheumatologic manifestations and tested for serum levels of TSH, Free T3 and T4, Anti-Nuclear Antibodies (ANAs) and Rheumatoid Factor (RF). SPSS software (version 16) and descriptive statistics were used for data analysis. Results: Nine males (14.8%) and 56 females (86.2%) with mean age of 38.81±1.44 years were studied. Overall, Rheumatologic manifestations were seen in 86.2 % (n=56). In this study, the most frequent rheumatologic manifestations were Carpal Tunnel Syndrome (36.1%) and Osteoarthritis (23%). Reynaud’s phenomenon (RP) (10.7%), Discopathy (8.9%), Fibromyalgia (5.3%), Myopathy (3.6%), Rheumatoid arthritis (3.6%) and trigger finger (3.6%) were other manifestations, respectively. Conclusion: In this region, there is a high frequency of rheumatologic manifestations in patients with ATDs. Thus, initial evaluation and regular checkings are recommended. PMID:25478383

  8. Urological Manifestations of Henoch-Schonlein Purpura: A Review

    PubMed Central

    Dalpiaz, Amanda; Schwamb, Richard; Miao, Yimei; Gonka, Jacquelyn; Walzter, Wayne; Khan, Sardar A.

    2015-01-01

    Henoch-Schonlein purpura (HSP) is an immune-mediated systemic vasculitis generally found in children. The standard manifestations of HSP are palpable purpura, arthritis, abdominal pain, and renal complications. Although less common, there are significant urological manifestations associated with HSP. The primary objective of this review is to encourage better understanding and management of HSP by emphasizing the common and rare manifestations of HSP, how they are diagnosed, and the latest treatment options for mild to severe complications. Medline searches of HSP and its urological manifestations were conducted along with searches on current diagnostic and treatment methods. Urological manifestations of HSP involve the kidney, ureter, bladder, prostate, scrotum, testicle, and penis. Diagnosis and management of HSP are not always clear due to differential diagnosis and diversity of symptom presentation. Treatment for HSP is mainly supportive and includes use of nonsteroidal anti-inflammatory drugs for pain relief. In more severe cases, glucocorticoids, methylprednisolone, plasmapheresis, and peritoneal and hemodialysis are reported successful. It is important to note different symptoms of HSP in order to distinguish HSP from other diseases. Early diagnosis may prevent severe complications. Treatment options vary from conservative to invasive depending on the severity of the disease and time frame of diagnosis. PMID:26889120

  9. Ocular Manifestations of Acquired Immunodeficiency Syndrome in Korea

    PubMed Central

    Kim, Sang Jin; Park, Sang Jun; Kim, Nam Joong; Jang, Hee-Chang; Oh, Myoung-don

    2012-01-01

    The clinical features of HIV/AIDS-related ocular manifestations in Korean patients were investigated in this study. Data on 200 consecutive Korean patients diagnosed with AIDS who visited the Seoul National University Hospital from January 2003 to June 2008 were reviewed. Fifty-seven patients (28.5%) had ocular manifestations, and they showed significantly lower CD4+ T cell count than patients without ocular manifestations. Among them, 23 (40.3%) patients showed retinal microvasculopathy, and 22 (38.5%) patients showed cytomegalovirus (CMV) retinitis. Other manifestations included retinal vein occlusion (n = 4), herpes zoster ophthalmicus (n = 4), syphilitic uveitis (n = 2), acute retinal necrosis (n = 1), and progressive outer retinal necrosis (n = 1). The mean CD4+ lymphocyte counts of the patients with retinal microvasculopathy and cytomegalovirus retinitis were 108.5 cells/µL and 69.4 cells/µL, respectively. In conclusion, ocular manifestations including CMV retinitis are common complications in Korean patients with AIDS even in the era of highly active anti-retroviral therapy. Compared to previous reports in western countries, prevalence of CMV retinitis is relatively low and CD4+ lymphocytes count at the time of diagnosis is relatively high. PMID:22563221

  10. Manifestations and complications of Mycoplasma pneumoniae disease: a review.

    PubMed Central

    Lind, K.

    1983-01-01

    Over the past 20 years the annual number of reports on extrapulmonary symptoms during Mycoplasma (M.) pneumoniae disease has increased. Clinical and epidemiological data indicate that symptoms from the skin and mucous membranes, from the central nervous system, from the heart, and perhaps from other organs as well are not quite uncommon manifestations of M. pneumoniae disease. Reports on unusual courses of the disease have also accumulated, including cases of severe respiratory symptoms, sometimes seen in patients with underlying disease or with a concomitant viral infection. Serious extrapulmonary manifestations have been common in fatal cases of M. pneumoniae disease. Some observations and experimental data on these manifestations and on the possible pathogenic mechanisms are dealt with. The conclusion is that such mechanisms are still largely unknown. PMID:6433567

  11. [Subcutaneous sarcoidosis as the first manifestation of systemic disease].

    PubMed

    Calzado, Leticia; Galera, Carmen María; Arrue, Itziar; Rodríguez-Peralto, José L; López, Sara; Guerra, Aurora; Vanaclocha, Francisco

    2005-01-01

    There are dermatological symptoms in up to 25% of patients with sarcoidosis, and the appearance of specific subcutaneous nodules as a manifestation of this entity is rare. They may even predate other manifestations of sarcoidosis. We present the case of a 38-year-old woman with asymptomatic subcutaneous nodules in the limbs, which corresponded to deep sarcoid granulomas in the histological study. She did not present with any extracutaneous indications. The imaging tests performed revealed right paratracheal adenopathies. This led to the diagnosis of sarcoidosis, in both its subcutaneous and pulmonary forms (stage I). Subcutaneous sarcoidosis is probably an underdiagnosed entity, as fewer than 40 cases are reflected in literature. Its value lies in the fact that it may be the first manifestation of extracutaneous or systemic sarcoidosis, which means that this form of sarcoidosis must be considered in the differential diagnosis of subcutaneous nodular lesions; close follow-up of these patients is also necessary.

  12. Extraintestinal manifestations of Helicobacter pylori: A concise review

    PubMed Central

    Wong, Frank; Rayner-Hartley, Erin; Byrne, Michael F

    2014-01-01

    Helicobacter pylori (H. pylori) infection has been clearly linked to peptic ulcer disease and some gastrointestinal malignancies. Increasing evidence demonstrates possible associations to disease states in other organ systems, known as the extraintestinal manifestations of H. pylori. Different conditions associated with H. pylori infection include those from hematologic, cardiopulmonary, metabolic, neurologic, and dermatologic systems. The aim of this article is to provide a concise review of the evidence that supports or refutes the associations of H. pylori and its proposed extraintestinal manifestations. Based on data from the literature, PUD, mucosal associated lymphoid tumors lymphoma, and gastric adenocarcinoma has well-established links. Current evidence most supports extraintestinal manifestations with H. pylori in immune thrombocytopenic purpura, iron deficiency anemia, urticaria, Parkinson’s, migraines and rosacea; however, there is still plausible link with other diseases that requires further research. PMID:25232230

  13. Comprehensive treatment of psoriatic arthritis: managing comorbidities and extraarticular manifestations.

    PubMed

    Ogdie, Alexis; Schwartzman, Sergio; Eder, Lihi; Maharaj, Ajesh B; Zisman, Devy; Raychaudhuri, Siba P; Reddy, Soumya M; Husni, Elaine

    2014-11-01

    Psoriatic arthritis (PsA) is an inflammatory arthritis associated with psoriasis that can lead to decreased health-related quality of life and permanent joint damage leading to functional decline. In addition to joint and skin manifestations, both psoriasis and PsA are associated with numerous comorbidities and extraarticular/cutaneous manifestations, which may influence the physician's choice of therapy. The objectives of this review are (1) to identify comorbidities in patients with PsA based on the available evidence; (2) to examine the effects of these comorbidities or extraarticular/cutaneous manifestation on the management of patients with PsA as well as the selection of therapy; and (3) to highlight research needs around comorbidities and treatment paradigms. This review is part of a treatment recommendations update initiated by the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA).

  14. Unusual neurologic manifestations occurring during dengue fever infection.

    PubMed

    Patey, O; Ollivaud, L; Breuil, J; Lafaix, C

    1993-06-01

    This is a report on dengue fever in two young patients in France that were infected in New Caledonia and Thailand. Both presented with unusual neurologic manifestations. The first patient developed a focal subarachnoid hemorrhage that was associated with transient thrombocytopenia. No neurologic vascular malformation was detected; a mild dengue hemorrhagic fever after a previous dengue infection was suspected. The second patient showed peripheral facial palsy one week after apyrexia without any other etiology except the dengue infection. This case was probably a postinfectious syndrome associated with dengue virus. Both patients recovered spontaneously. The circumstances of the neurologic manifestations in these patients may be attributed to the dengue fever virus. However, although neurologic complications reported for dengue fever are unusual, it is reasonable to consider these manifestations as being due to immunopathologic consequences.

  15. Use of Biologic Agents in Ocular Manifestations of Rheumatic Disease

    PubMed Central

    Kraus, Courtney L.; Culican, Susan M.

    2012-01-01

    Biologic agents have dramatically shifted the treatment paradigm for rheumatic disease. Use of these agents can decrease disease burden, allow the patient to be weaned from corticosteroids, and reduce the likelihood of relapse. Eye disease associated with rheumatic conditions may present with a wide range of signs and symptoms. This coexisting pathology should not be overlooked and should be considered a reason for initiation or continuation of biologic therapy. Additionally, many of the ocular manifestations of rheumatic disease respond preferentially to specific targeting molecules. This paper summarizes the available studies on the use, efficacy, and safety of biologic agents in the treatment of ocular manifestations of rheumatic disease. PMID:22229035

  16. Advancements in Pharmacotherapy for Noncancerous Manifestations of HPV.

    PubMed

    Kollipara, Ramya; Ekhlassi, Erfon; Downing, Christopher; Guidry, Jacqueline; Lee, Michael; Tyring, Stephen K

    2015-04-24

    Human papillomavirus (HPV) is the most common sexually transmitted disease. Via infection of the basal epithelial cells, HPV causes numerous malignancies and noncancerous cutaneous manifestations. Noncancerous cutaneous manifestations of HPV, including common, plantar, plane, and anogenital warts, are among the most common reasons for an office visit. Although there are various therapies available, they are notoriously difficult to treat. HPV treatments can be grouped into destructive (cantharidin, salicylic acid), virucidal (cidofovir, interferon-α), antimitotic (bleomycin, podophyllotoxin, 5-fluorouracil), immunotherapy (Candida antigen, contact allergen immunotherapy, imiquimod) or miscellaneous (trichloroacetic acid, polyphenon E). The mechanism of action, recent efficacy data, safety profile and recommended regimen for each of these treatment modalities is discussed.

  17. Oral Manifestations and Molecular Basis of Oral Genodermatoses: A Review

    PubMed Central

    Shilpasree, A.S.; Chaudhary, Meenakshi

    2016-01-01

    Genodermatoses refers to group of inherited monogenic disorders with skin manifestations. Many of these disorders are rare and also have oral manifestations, called oral genodermatoses. This article provides a focused review of molecular basis of important genodermatoses that affects the oral cavity and also have prominent associated dermatologic features. In several conditions discussed here, the oral findings are distinct and may provide the first clue of an underlying genetic diagnosis. The article also emphasises on the prenatal diagnosis, genetic counselling and the treatment oral genodermatoses. PMID:27437377

  18. Emerging infectious diseases with cutaneous manifestations: Viral and bacterial infections.

    PubMed

    Nawas, Zeena Y; Tong, Yun; Kollipara, Ramya; Peranteau, Andrew J; Woc-Colburn, Laila; Yan, Albert C; Lupi, Omar; Tyring, Stephen K

    2016-07-01

    Given increased international travel, immigration, and climate change, bacterial and viral infections that were once unrecognized or uncommon are being seen more frequently in the Western Hemisphere. A delay in diagnosis and treatment of these diseases can lead to significant patient morbidity and mortality. However, the diagnosis and management of these infections is fraught with a lack of consistency because there is a dearth of dermatology literature on the cutaneous manifestations of these infections. We review the epidemiology, cutaneous manifestations, diagnosis, and management of these emerging bacterial and viral diseases.

  19. Oral Manifestations and Molecular Basis of Oral Genodermatoses: A Review.

    PubMed

    Kumar, Kiran; Shilpasree, A S; Chaudhary, Meenakshi

    2016-05-01

    Genodermatoses refers to group of inherited monogenic disorders with skin manifestations. Many of these disorders are rare and also have oral manifestations, called oral genodermatoses. This article provides a focused review of molecular basis of important genodermatoses that affects the oral cavity and also have prominent associated dermatologic features. In several conditions discussed here, the oral findings are distinct and may provide the first clue of an underlying genetic diagnosis. The article also emphasises on the prenatal diagnosis, genetic counselling and the treatment oral genodermatoses. PMID:27437377

  20. Manifesting color-kinematics duality in the scattering equation formalism

    NASA Astrophysics Data System (ADS)

    Bjerrum-Bohr, N. E. J.; Bourjaily, Jacob L.; Damgaard, Poul H.; Feng, Bo

    2016-09-01

    We prove that the scattering equation formalism for Yang-Mills amplitudes can be used to make manifest the theory's color-kinematics duality. This is achieved through a concrete reduction algorithm which renders this duality manifest term-by-term. The reduction follows from the recently derived set of identities for amplitudes expressed in the scattering equation formalism that are analogous to monodromy relations in string theory. A byproduct of our algorithm is a generalization of the identities among gravity and Yang-Mills amplitudes.

  1. Progressive kidney failure as the sole manifestation of extrapulmonary sarcoidosis.

    PubMed

    Sethi, Supreet; Relia, Nitin; Syal, Gaurav; Kaushik, Chhavi; Gokden, Neriman; Malik, Ahmad B

    2013-09-01

    Sarcoidosis is a chronic multisystem disorder characterized by an accumulation of T lymphocytes and mononuclear phagocytes, non-caseating epitheliod granulomas and derangement of normal tissue architecture in affected organs. Sarcoidosis can affect any organ system, however approximately 90% of patients with sarcoidosis have pulmonary, lymph node, cutaneous or ocular manifestations. Renal involvement in sarcoidosis is rare and clinically significant renal dysfunction even less common. We present a case of isolated renal sarcoidosis which manifested with progressively worsening renal function and hypercalcemia. A systematic diagnostic approach with pertinent laboratory studies, imaging and renal biopsy elucidated the diagnosis of renal sarcoidosis without any evidence of systemic involvement.

  2. Neurologic and Head and Neck Manifestations of Sickle Cell Disease.

    PubMed

    Steven, Andrew; Raghavan, Prashant; Rath, Tanya J; Gandhi, Dheeraj

    2016-08-01

    Sickle cell disease is a common, inherited disordered characterized by chronic hemolytic anemia with repetitive episodes of vasoocclusion resulting from deformed red blood cells. This article reviews the most significant neurologic and head and neck manifestations of this disease. PMID:27443997

  3. Self-Organization in the Manifestations of Youth Extremism

    ERIC Educational Resources Information Center

    Zubok, Iu. A.; Chuprov, V. I.

    2011-01-01

    The analysis of the nature of youth extremism has shown that there is a connection between the extremist tendency ["ekstremal'nost'"] that is an essential property of young people, on the one hand, and extremist manifestations that come about in that community under certain conditions. These conditions include external ones (the economic,…

  4. 40 CFR 263.20 - The manifest system.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... H of 40 CFR part 262, a transporter may not accept such waste from a primary exporter or other... manifest (or shipping paper for exports by water (bulk shipment)). For exports of hazardous waste subject to the requirements of subpart H of 40 CFR part 262, a transporter may not accept hazardous...

  5. 40 CFR 264.71 - Use of manifest system.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 264.71 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES (CONTINUED) STANDARDS FOR OWNERS AND OPERATORS OF HAZARDOUS WASTE TREATMENT, STORAGE, AND DISPOSAL FACILITIES Manifest... 75 FR 1260, Jan. 8, 2010. (a)(1) If a facility receives hazardous waste accompanied by a...

  6. [Acute encephalitis. Neuropsychiatric manifestations as expression of influenza virus infection].

    PubMed

    Moreno-Flagge, Noris; Bayard, Vicente; Quirós, Evelia; Alonso, Tomás

    2009-01-01

    The aim is to review the encephalitis in infants and adolescents as well as its etiology, clinical manifestation, epidemiology, physiopathology, diagnostic methods and treatment, and the neuropsyquiatric signs appearing an influenza epidemy. Encephalitis is an inflammation of the central nervous system (CNS) which involves the brain. The clinical manifestations usually are: headache, fever and confusional stage. It could also be manifested as seizures, personality changes, or psiqyiatric symptoms. The clinical manifestations are related to the virus and the cell type affected in the brain. A meningitis or encephalopathy need to be ruled out. It could be present as an epidemic or isolated form, beeing this the most frequent form. It could be produced by a great variety of infections agents including virus, bacterias, fungal and parasitic. Viral causes are herpesvirus, arbovirus, rabies and enterovirus. Bacterias such as Borrelia burgdorferi, Rickettsia and Mycoplasma neumoniae. Some fungal causes are: Coccidioides immitis and Histoplasma capsulatum. More than 100 agents are related to encephalitis. The diagnosis of encephalitis is a challenge for the clinician and its infectious etiology is clear in only 40 to 70% of all cases. The diagnosis of encephalitis can be established with absolute certainty only by the microscopic examination of brain tissue. Epidemiology is related to age of the patients, geographic area, season, weather or the host immune system. Early intervention can reduce the mortality rate and sequels. We describe four patients with encephalitis and neuropsychiatric symptoms during an influenza epidemic.

  7. 19 CFR 123.22 - In-transit manifest.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... sealed with Customs seals shall be “miscellaneous shipments.” (3) When an in-transit rail shipment will... 19 Customs Duties 1 2013-04-01 2013-04-01 false In-transit manifest. 123.22 Section 123.22 Customs... CBP RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22...

  8. 19 CFR 123.22 - In-transit manifest.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... sealed with Customs seals shall be “miscellaneous shipments.” (3) When an in-transit rail shipment will... 19 Customs Duties 1 2012-04-01 2012-04-01 false In-transit manifest. 123.22 Section 123.22 Customs... CBP RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22...

  9. 19 CFR 123.22 - In-transit manifest.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... sealed with Customs seals shall be “miscellaneous shipments.” (3) When an in-transit rail shipment will... 19 Customs Duties 1 2011-04-01 2011-04-01 false In-transit manifest. 123.22 Section 123.22 Customs... CBP RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.22...

  10. Links between Early Attachment Experiences and Manifestations of Spirituality

    ERIC Educational Resources Information Center

    Surr, John

    2011-01-01

    This essay reviews recent research about infant attachment, then discusses the implications of this research as they relate to the following specific manifestations of children's spirituality: faith, wonder, relational consciousness, flow (as in a sense of wholeness or unity), and compassion, in the light of other research on children's…

  11. Testing Manifest Monotonicity Using Order-Constrained Statistical Inference

    ERIC Educational Resources Information Center

    Tijmstra, Jesper; Hessen, David J.; van der Heijden, Peter G. M.; Sijtsma, Klaas

    2013-01-01

    Most dichotomous item response models share the assumption of latent monotonicity, which states that the probability of a positive response to an item is a nondecreasing function of a latent variable intended to be measured. Latent monotonicity cannot be evaluated directly, but it implies manifest monotonicity across a variety of observed scores,…

  12. 40 CFR 264.71 - Use of manifest system.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ...) Within three (3) working days of the receipt of a shipment subject to 40 CFR part 262, subpart H, the... 40 Protection of Environment 26 2011-07-01 2011-07-01 false Use of manifest system. 264.71 Section 264.71 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES...

  13. [Lupus vulgaris manifestation as a destructive nose and facial tumor].

    PubMed

    Haller, D; Reisser, C

    2009-04-01

    Lupus vulgaris is the most frequent manifestation of cutaneous tuberculosis, but in Europe it is limited to isolated cases. Mainly immunocompetent individuals are affected by this result of an endogenous reinfection on a lymphogenous-less frequently hematogenous-pathway. Lupus vulgaris has been observed to develop in more than 50% of all patients who already suffer from other manifestations of tuberculosis. The development of a squamous cell carcinoma in the lupus vulgaris is a rare complication; therefore, lupus vulgaris is deemed a facultative precancerosis.A 68-year-old female Serbo-Croatian patient presented with an extensive ulcerative nose and facial tumor. Her anamnesis included a squamous cell carcinoma of the nose that had been excised alio loco 3 years before. Further examinations revealed enlarged cervical lymphoma on both sides, and pulmonary metastases were also suspected. The tumor biopsy revealed a necrotic, granulomatous inflammation. No acid-fast rods were seen on Ziehl-Neelsen stain. The tuberculous origin of this ulcerative skin tumor-the lupus vulgaris-as an endogenous reinfection of pulmonary tuberculosis manifestation was confirmed by the detection of Mycobacterium tuberculosis DNA in polymerase chain reaction and the growth of Mycobacterium tuberculosis colonies in the bacterial culture (skin biopsy and bronchial secretion). The skin tumor as well as the pulmonary manifestation were successfully treated with combined tuberculostatic therapy and showed a dramatic response within 3 months.

  14. Neuropsychiatric manifestations in systemic lupus erythematosus: epidemiology, pathophysiology and management.

    PubMed

    Postal, Mariana; Costallat, Lilian T L; Appenzeller, Simone

    2011-09-01

    Systemic lupus erythematosus (SLE) is a relapsing-remitting autoimmune disease with CNS involvement occurring in up to 75% of patients. However, the frequency of neuropsychiatric manifestations in SLE studies varies widely, depending on the type of manifestations included and the method used for evaluation. CNS involvement may be considered primary if directly related to SLE activity in the CNS or secondary when related to treatment, infections, metabolic abnormalities or other systemic manifestations such as uraemia and hypertension. The pathogenesis of neuropsychiatric SLE is as yet unknown, though numerous autoantibodies and cytokines have been suggested as possible mediators. However, independent of the aetiology of the insult, the final common pathway in neuropsychiatric SLE is the involvement of the cerebral microvasculature. The diagnosis of primary CNS involvement by SLE is often difficult, as both focal and diffuse manifestations may occur and there is no gold standard for diagnosis. A high index of clinical suspicion, in addition to laboratory and neuroimaging findings may support the diagnosis. Treatment is mostly empirical, although one randomized controlled trial has shown that cyclophosphamide in addition to methylprednisolone is superior to methylprednisolone alone in severe neuropsychiatric SLE.

  15. 10 CFR 20.2006 - Transfer for disposal and manifests.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ....2006 Energy NUCLEAR REGULATORY COMMISSION STANDARDS FOR PROTECTION AGAINST RADIATION Waste Disposal... 10 CFR part 20 are designed to— (1) Control transfers of low-level radioactive waste by any waste... waste land disposal facility (as defined in part 61 of this chapter); (2) Establish a manifest...

  16. 10 CFR 20.2006 - Transfer for disposal and manifests.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ....2006 Energy NUCLEAR REGULATORY COMMISSION STANDARDS FOR PROTECTION AGAINST RADIATION Waste Disposal... 10 CFR part 20 are designed to— (1) Control transfers of low-level radioactive waste by any waste... waste land disposal facility (as defined in part 61 of this chapter); (2) Establish a manifest...

  17. 10 CFR 20.2006 - Transfer for disposal and manifests.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ....2006 Energy NUCLEAR REGULATORY COMMISSION STANDARDS FOR PROTECTION AGAINST RADIATION Waste Disposal... 10 CFR part 20 are designed to— (1) Control transfers of low-level radioactive waste by any waste... waste land disposal facility (as defined in part 61 of this chapter); (2) Establish a manifest...

  18. 10 CFR 20.2006 - Transfer for disposal and manifests.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ....2006 Energy NUCLEAR REGULATORY COMMISSION STANDARDS FOR PROTECTION AGAINST RADIATION Waste Disposal... 10 CFR part 20 are designed to— (1) Control transfers of low-level radioactive waste by any waste... waste land disposal facility (as defined in part 61 of this chapter); (2) Establish a manifest...

  19. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 8 Aliens and Nationality 1 2014-01-01 2014-01-01 false Arrival manifests and lists. 251.1 Section 251.1 Aliens and Nationality DEPARTMENT OF HOMELAND SECURITY IMMIGRATION REGULATIONS ARRIVAL AND... or agent of the vessel shall indicate in writing immediately below the name of the last alien...

  20. 40 CFR 761.214 - Retention of manifest records.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... Section 761.214 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.214 Retention of manifest records. (a)(1) A generator... receives a signed copy from the designated facility which received the PCB waste. This signed copy must...

  1. 40 CFR 761.211 - Manifest system-Transporter requirements.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ...) TOXIC SUBSTANCES CONTROL ACT POLYCHLORINATED BIPHENYLS (PCBs) MANUFACTURING, PROCESSING, DISTRIBUTION IN COMMERCE, AND USE PROHIBITIONS PCB Waste Disposal Records and Reports § 761.211 Manifest system—Transporter requirements. (a)(1) A transporter shall not accept PCB waste from a generator unless it is accompanied by...

  2. 40 CFR 761.211 - Manifest system-Transporter requirements.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ...) TOXIC SUBSTANCES CONTROL ACT POLYCHLORINATED BIPHENYLS (PCBs) MANUFACTURING, PROCESSING, DISTRIBUTION IN COMMERCE, AND USE PROHIBITIONS PCB Waste Disposal Records and Reports § 761.211 Manifest system—Transporter requirements. (a)(1) A transporter shall not accept PCB waste from a generator unless it is accompanied by...

  3. 40 CFR 761.214 - Retention of manifest records.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... Section 761.214 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) TOXIC SUBSTANCES... PROHIBITIONS PCB Waste Disposal Records and Reports § 761.214 Retention of manifest records. (a)(1) A generator... receives a signed copy from the designated facility which received the PCB waste. This signed copy must...

  4. The Development and Manifestation of Altruistic Caring: A Qualitative Inquiry

    ERIC Educational Resources Information Center

    Curry, Jennifer R.; Smith, Heather L.; Robinson, Edward H., III

    2009-01-01

    Qualitative, phenomenological research provides rich information about the constructive, life span perspectives of the manifestation and development of altruism. Using an interpretive phenomenological approach, this study investigated "altruism" as described by 34 older persons in a continuing care retirement community. The findings identified 13…

  5. Space Launch System Co-Manifested Payload Options for Habitation

    NASA Technical Reports Server (NTRS)

    Smitherman, David

    2015-01-01

    The Space Launch System (SLS) has a co-manifested payload capability that will grow over time as the launch vehicle matures and planned upgrades are implemented. The final configuration is planned to be capable of inserting a payload greater than 10 metric tons (mt) into a trans-lunar injection trajectory along with the crew in the Orion capsule and its service module. The co-manifested payload is located below the Orion and its service module in a 10 m high fairing similar to the way the Saturn launch vehicle carried the lunar lander below the Apollo command and service modules. Various approaches that utilize this comanifested payload capability to build up infrastructure in deep space have been explored in support of future asteroid, lunar, and Mars mission scenarios. This paper reports on the findings of the Advanced Concepts Office study team at NASA Marshall Space Flight Center (MSFC) working with the Advanced Exploration Systems Program on the Exploration Augmentation Module Project. It includes some of the possible options for habitation in the co-manifested payload volume of the SLS. Findings include a set of module designs that can be developed in 10 mt increments to support these co-manifested payload missions along with a comparison of this approach to a large-module payload flight configuration for the SLS.

  6. Acute meningoencephalitis as the sole manifestation of Q fever.

    PubMed

    Guerrero, M; Gutierrez, J; Carnero, C; Gonzalez-Maldonado, R; Maroto, C

    1993-01-01

    The case of a 25-year old man who presented with meningoencephalitis as the sole clinical manifestation of Q fever is described. Serological studies revealed the presence of IgM and IgG antibodies to Coxiella burnetii. The patient responded favourably to a ten-day course of i.v. ceftriaxone and was discharged without any neurological sequelae.

  7. 19 CFR 123.25 - Certification and disposition of manifests.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...; DEPARTMENT OF THE TREASURY CUSTOMS RELATIONS WITH CANADA AND MEXICO Shipments in Transit Through Canada or Mexico § 123.25 Certification and disposition of manifests. (a) Certification. Conveyances proceeding... required when the merchandise is to be transshipped in Canada or Mexico under Customs supervision shall...

  8. Manifestations of CP Violation in the MSSM Higgs Sector

    SciTech Connect

    Lee, Jae Sik

    2008-11-23

    We demonstrate how CP violation manifests itself in the Higgs sector of the minimal supersymmetric extension of the Standard Model (MSSM). Starting with a brief introduction to CP violation in the MSSM and its effects on the Higgs sector, we discuss some phenomenological aspects of the Higgs sector CP violation based on the two scenarios called CPX and Trimixing.

  9. 77 FR 54863 - Polychlorinated Biphenyls (PCBs): Revisions to Manifesting Regulations

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-09-06

    ... AGENCY 40 CFR Part 761 ] RIN 2050-AG71 Polychlorinated Biphenyls (PCBs): Revisions to Manifesting... Protection Agency (``EPA'' or ``the Agency'') is issuing this proposed rule to update and clarify several... Toxic Substances Control Act (TSCA). Today's changes are to match, as much as possible, the...

  10. 40 CFR 263.20 - The manifest system.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... CFR 177.817, a hazardous waste transporter must carry one printed copy of the electronic manifest on... H of 40 CFR part 262, a transporter may not accept such waste from a primary exporter or other... to the requirements of subpart H of 40 CFR part 262, a transporter may not accept hazardous...

  11. 40 CFR 265.71 - Use of manifest system.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... or obtaining a valid and enforceable electronic signature within the meaning of 40 CFR 262.25. (2... date of delivery of the waste. (j) Imposition of user fee for electronic manifest use. An owner or... 265.71 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES...

  12. Reliability Generalization of the Revised Children's Manifest Anxiety Scale

    ERIC Educational Resources Information Center

    Ryngala, Donna J.; Shields, Alan L.; Caruso, John C.

    2005-01-01

    A reliability generalization of the Revised Children's Manifest Anxiety Scale (RCMAS) was conducted using the normative sample. The RCMAS consists of a Total Anxiety scale as well as four subscales. Results suggest that the Total Anxiety scores are typically reliable (median across 48 samples = .81). Subscale scores were less reliable: The median…

  13. 49 CFR 172.205 - Hazardous waste manifest.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 49 Transportation 2 2010-10-01 2010-10-01 false Hazardous waste manifest. 172.205 Section 172.205 Transportation Other Regulations Relating to Transportation PIPELINE AND HAZARDOUS MATERIALS SAFETY ADMINISTRATION, DEPARTMENT OF TRANSPORTATION HAZARDOUS MATERIALS REGULATIONS HAZARDOUS MATERIALS TABLE, SPECIAL PROVISIONS, HAZARDOUS...

  14. Bleeding Ectopic Varices as the First Manifestation of Portal Hypertension

    PubMed Central

    Sharma, Brij; Raina, Sujeet; Sharma, Rajesh

    2014-01-01

    Ectopic varices are defined as dilated portosystemic collateral veins in locations other than the gastroesophageal region. We present a case of recurrent upper gastrointestinal bleeding as the first manifestation of portal hypertension. We diagnosed ectopic duodenal varices without gastroesophageal varices on upper GI endoscopy and extrahepatic portal venous obstruction (EHPVO) on CT angiography and managed this case. PMID:25374725

  15. 76 FR 36480 - Hazardous Waste Manifest Printing Specifications Correction Rule

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-06-22

    ... indicate that red ink, as well as other distinct colors, or other methods to distinguish the copy... would afford authorized manifest form printers ] greater flexibility in complying with the Federal... in red ink. In the ``Rules and Regulations'' section of this Federal Register, EPA is making...

  16. Social Support in Elderly Nursing Home Populations: Manifestations and Influences

    ERIC Educational Resources Information Center

    Rash, Elizabeth M.

    2007-01-01

    The purpose of this study was to describe the characteristics of social support and the influencing factors on social support in nursing home environments. Observations and staff questionnaires from two central Florida nursing homes were used in this grounded theory study to answer the following questions: (1) How is social support manifested? and…

  17. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... be transporting dry bulk cargo that qualifies as hazardous. All tankers qualify for the hazardous... vessel is a qualifying tanker. (B) If the vessel is transporting dry bulk hazardous cargo, the master or... immigration officer the dangerous cargo manifest that is signed by the master or an authorized...

  18. 8 CFR 251.1 - Arrival manifests and lists.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... be transporting dry bulk cargo that qualifies as hazardous. All tankers qualify for the hazardous... vessel is a qualifying tanker. (B) If the vessel is transporting dry bulk hazardous cargo, the master or... immigration officer the dangerous cargo manifest that is signed by the master or an authorized...

  19. 46 CFR 148.70 - Dangerous cargo manifest; general.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ..., each vessel transporting materials listed in Table 148.10 of this part must have a dangerous cargo... 46 Shipping 5 2011-10-01 2011-10-01 false Dangerous cargo manifest; general. 148.70 Section 148.70 Shipping COAST GUARD, DEPARTMENT OF HOMELAND SECURITY (CONTINUED) DANGEROUS CARGOES CARRIAGE OF BULK...

  20. 46 CFR 148.70 - Dangerous cargo manifest; general.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ..., each vessel transporting materials listed in Table 148.10 of this part must have a dangerous cargo... 46 Shipping 5 2014-10-01 2014-10-01 false Dangerous cargo manifest; general. 148.70 Section 148.70 Shipping COAST GUARD, DEPARTMENT OF HOMELAND SECURITY (CONTINUED) DANGEROUS CARGOES CARRIAGE OF BULK...

  1. 46 CFR 148.70 - Dangerous cargo manifest; general.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ..., each vessel transporting materials listed in Table 148.10 of this part must have a dangerous cargo... 46 Shipping 5 2012-10-01 2012-10-01 false Dangerous cargo manifest; general. 148.70 Section 148.70 Shipping COAST GUARD, DEPARTMENT OF HOMELAND SECURITY (CONTINUED) DANGEROUS CARGOES CARRIAGE OF BULK...

  2. 46 CFR 148.70 - Dangerous cargo manifest; general.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ..., each vessel transporting materials listed in Table 148.10 of this part must have a dangerous cargo... 46 Shipping 5 2013-10-01 2013-10-01 false Dangerous cargo manifest; general. 148.70 Section 148.70 Shipping COAST GUARD, DEPARTMENT OF HOMELAND SECURITY (CONTINUED) DANGEROUS CARGOES CARRIAGE OF BULK...

  3. Ocular manifestation of rheumatoid arthritis-different forms and frequency.

    PubMed

    Zlatanović, Gordana; Veselinović, Dragan; Cekić, Sonja; Zivković, Maja; Dorđević-Jocić, Jasmina; Zlatanović, Marko

    2010-11-01

    Rheumatoid arthritis (RA) is a systemic inflammatory disease associated with a number of extra-articular organ manifestations. Ocular manifestations involved with RA are keratoconjunctivitis sicca, episcleritis, scleritis , corneal changes, and retinal vasculitus. The etiopathogenesis of this autoimmune disorder is still unknown. Aim of our study was to present different ocular manifestations of RA and their frequency. We have examined 691 patient with the diagnoses of RA. All examined patients were in I or II stage of the disease according to criteria of The American College of Rheumatology. Ophthalmological exam obtained: visual acuity by Snellen sings, biomicroscopy of anterior segment, Schirmer test, tear break-up time (BUT), applanation tonometry and indirect ophthalmoscopy. In all patients with retinal vasculitis fotofundus and in indicated cases fluorescein angiography was preformed. The most common manifestation of ocular involvement was keratoconjunctivitis sicca. Episcleritis was diagnosed in 5.06% patients with RA, while scleritis was present in 2.06% of patients. Diffuse scleritis was present in one patient, while nodular was present in 13 patients. There were no patients with posterior or necrotizing scleritis among examined patients. Sclerosing keratitis was diagnosed in 11 female patients. It is characterized with peripheral thickening and opacification of the stroma adjacent to the site of inflammation. Posterior scleritis or scleromalacia of cornea was not present in our patients, because all of them were in I or II stage of disease. Retinal vasculitis was present in three patients, two male and one female patient (0.45%). Ocular manifestation was present in 27.2% of patients. Women were more affected.

  4. Radiologic manifestations in the musculoskeletal system of miscellaneous endocrine disorders.

    PubMed

    Chew, F S

    1991-01-01

    The manifestations of endocrine derangements in the musculoskeletal system in infancy and childhood are disturbances in growth and maturation and in adulthood are disturbances in maintenance and metabolism. Hypercortisolism during skeletal immaturity suppresses growth. In the adult, hypercortisolism leads to osteoporosis, osteonecrosis, and muscle wasting. Deficiency of growth hormone during skeletal development results in short stature. An excess of growth hormone in a skeletally immature individual results in gigantism, an excess in a skeletally mature individual results in acromegaly. Patients with gigantism have extreme height with normal body proportions. Musculoskeletal manifestations of acromegaly include soft-tissue thickening, vertebral body enlargement, characteristic hand and foot changes, and enthesal bony proliferation. Hyperthyroidism causes catabolism of protein and loss of connective tissue, which manifest as muscle wasting. Deficient levels of thyroid hormone cause defects in growth and development. Severe growth retardation from congenital hypothyroidism is rare because neonatal screening recognizes the disorder and leads to early treatment. The skeletal manifestation of hypergonadism in children is precocious growth and early skeletal maturation. Although the initial precocious growth spurt results in a tall child, early closure of the growth plates results in a short adult. Hypogonadism in the prepubertal child results in delayed adolescence and delayed skeletal maturation. Diabetes mellitus in childhood results in decreased growth, a phenomenon presumed to be secondary to nutritional abnormalities. Generalized osteoporosis and short stature are common. In the adult, generalized osteoporosis may accompany insulin-dependent diabetes mellitus if obesity is absent. Calcification of interdigital arteries of the foot is common in diabetics and uncommon in other conditions. Additional skeletal manifestations relate to complications of diabetes such as

  5. 19 CFR 122.113 - Form for transit air cargo manifest procedures.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 19 Customs Duties 1 2010-04-01 2010-04-01 false Form for transit air cargo manifest procedures... SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures. A manifest on Customs Form 7509 is...

  6. 19 CFR 122.113 - Form for transit air cargo manifest procedures.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 19 Customs Duties 1 2011-04-01 2011-04-01 false Form for transit air cargo manifest procedures... SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures. A manifest on Customs Form 7509 is...

  7. 19 CFR 122.113 - Form for transit air cargo manifest procedures.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 19 Customs Duties 1 2012-04-01 2012-04-01 false Form for transit air cargo manifest procedures... SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures. A manifest on Customs Form 7509 is...

  8. 19 CFR 122.113 - Form for transit air cargo manifest procedures.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 19 Customs Duties 1 2014-04-01 2014-04-01 false Form for transit air cargo manifest procedures... SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures. A manifest on Customs Form 7509 is...

  9. 19 CFR 122.113 - Form for transit air cargo manifest procedures.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 19 Customs Duties 1 2013-04-01 2013-04-01 false Form for transit air cargo manifest procedures... SECURITY; DEPARTMENT OF THE TREASURY AIR COMMERCE REGULATIONS Transit Air Cargo Manifest (TACM) Procedures § 122.113 Form for transit air cargo manifest procedures. A manifest on Customs Form 7509 is...

  10. What type of different clinical manifestations can cardiac sarcoidosis present?

    PubMed

    Şentürk, Ayşegül; Maraş, Yüksel; Argüder, Emine; Karalezli, Ayşegül; Hasanoğlu, H Canan; Öğüt, Tuba; Baştuğ, Serdal; Karabekir, Ercan

    2015-06-01

    Cardiac sarcoidosis is an infiltrative, granulomatous inflammatory disease of the myocardium. Generally, it can be difficult to diagnose cardiac sarcoidosis clinically because of the non-specific nature of its clinical manifestations. This property can be based on the presence of any clinical evidence of sarcoidosis in the other organs. We present two cases of cardiac sarcoidosis so as to demonstrate its different clinical manifestations. The first patient displayed no cardiac symptoms; the electrocardiogram showed an incidental right bundle branch block. Her cardiac magnetic resonance imaging (CMRI) revealed late-phase opaque material enhancement involving the inferior and inferoseptal segment of the left ventricle. The second patient was severely symptomatic in terms of cardiac involvement, and a transthoracic echocardiogram revealed global hypokinesia and septal brightness; his ejection fraction decreased to 45%. These cases highlighted the challenges encountered in the diagnosis and treatment of cardiac sarcoidosis. CMRI should be considered in all patients who have suspected findings for cardiac involvement.

  11. [Clinical and radiologic manifestations of larval paragonimiasis in children].

    PubMed

    Perelśhteĭn, N N; Sipukhin, Ia M; Siuzeva, N A; Apukhtina, T P

    1991-01-01

    Investigation of 60 children with acute types and 45 children with latent types of larval paragonimiasis (LP) has revealed various clinical and x-ray manifestations of this disease, spread in the southern Primorski Territory. Three syndromes of this disease were singled out: toxico-allergic, abdominal and pulmonary. X-ray investigation showed characteristic LP symptoms: exudate in the pleural cavity, thickening of the wall, diaphragmatic and interlobular pleura, sometimes--pneumothorax. Pathology of the bronchopulmonary system manifested itself in focal and infiltrative shadows, enhanced and deformed lung markings, and bullous inflation. Diagnosis and differential diagnosis must be based on analysis of clinical and x-ray data and the results of serological reactions.

  12. Advancements in Pharmacotherapy for Noncancerous Manifestations of HPV

    PubMed Central

    Kollipara, Ramya; Ekhlassi, Erfon; Downing, Christopher; Guidry, Jacqueline; Lee, Michael; Tyring, Stephen K.

    2015-01-01

    Human papillomavirus (HPV) is the most common sexually transmitted disease. Via infection of the basal epithelial cells, HPV causes numerous malignancies and noncancerous cutaneous manifestations. Noncancerous cutaneous manifestations of HPV, including common, plantar, plane, and anogenital warts, are among the most common reasons for an office visit. Although there are various therapies available, they are notoriously difficult to treat. HPV treatments can be grouped into destructive (cantharidin, salicylic acid), virucidal (cidofovir, interferon-α), antimitotic (bleomycin, podophyllotoxin, 5-fluorouracil), immunotherapy (Candida antigen, contact allergen immunotherapy, imiquimod) or miscellaneous (trichloroacetic acid, polyphenon E). The mechanism of action, recent efficacy data, safety profile and recommended regimen for each of these treatment modalities is discussed. PMID:26239450

  13. Hypomelanosis of Ito: a manifestation of mosaicism or chimerism.

    PubMed Central

    Donnai, D; Read, A P; McKeown, C; Andrews, T

    1988-01-01

    We describe three patients with the cutaneous manifestations of hypomelanosis of Ito. Two, with unusual abnormalities of their toes, had a mixture of diploid and triploid cells in cultured skin fibroblasts. The published clinical descriptions of hypomelanosis of Ito and diploid-triploid mosaicism are reviewed. Chromosome heteromorphisms, HLA types, and DNA fingerprints were studied in an attempt to elucidate the origin of the disease in our patients. We conclude that hypomelanosis of Ito is a manifestation of a heterogeneous group of disorders, the common factor being the presence of two genetically different cell lines. It can result from chromosomal mosaicism or chimerism, from a postzygotic mutation, or from X inactivation. The risk of recurrence is negligible if the proband is a male; if the proband is female the risk is also low but an X linked mutation must be considered. Images PMID:3236362

  14. Progressive outer retinal necrosis: manifestation of human immunodeficiency virus infection.

    PubMed

    Lo, Phey Feng; Lim, Rongxuan; Antonakis, Serafeim N; Almeida, Goncalo C

    2015-01-01

    We present the case of a 54-year-old man who developed progressive outer retinal necrosis (PORN) as an initial manifestation of HIV infection without any significant risk factors for infection with HIV. PORN is usually found as a manifestation of known AIDS late in the disease. Our patient presented with transient visual loss followed by decrease in visual acuity and facial rash. Subsequent investigation revealed anterior chamber tap positive for varicella zoster virus (VZV), as well as HIV positivity, with an initial CD4 count of 48 cells/µL. Systemic and intravitreal antivirals against VZV, and highly active antiretroviral therapy against HIV were started, which halted further progression of retinal necrosis. This case highlights the importance of suspecting PORN where there is a rapidly progressive retinitis, and also testing the patient for HIV, so appropriate treatment can be started. PMID:25948844

  15. Atypical clinical manifestations of multiple endocrine neoplasia type 1 syndrome.

    PubMed

    Krysiak, Robert; Kajdaniuk, Dariusz; Marek, Bogdan; Okopień, Bogusław

    2009-03-01

    Multiple endocrine neoplasia type 1 (MEN1) is a hereditary tumor syndrome characterized by a genetic predisposition to develop a variety of neuroendocrine tumors and hormone excess syndromes. The major components of MEN1 are hyperparathyroidism due to multiple parathyroid adenomas or hyperplasia, duodenopancreatic neuroendocrine tumors and pituitary adenomas, most often producing prolactin. Physicians' inadequate knowledge of this clinical entity and sometimes its atypical presentation result in a probable significant underdiagnosis of MEN1. This describes the case of a 65-year-old female in whom primary hyperparathyroidism, limited to only one parathyroid gland, was preceded by acromegaly that was diagnosed 23 years earlier. This case shows that MEN1 manifests itself even in older groups and hyperparathyroidism may not be the first symptom of this syndrome. Therefore, we believe that all subjects who, regardless of age, gender and initial manifestation present with whichever the major symptom should be followed up regularly for the early detection of MEN1. PMID:19514648

  16. Extrahepatic Manifestations of Hepatitis C Infection: Navigating CHASM

    PubMed Central

    Sherman, Amy C.; Sherman, Kenneth E.

    2015-01-01

    Purpose of review This article describes the importance of extra-hepatic systemic manifestations of chronic hepatitis C virus (HCV) infection. Recent findings While most HCV literature focuses on liver injury and fibrosis progression, a spectrum of systemic disease processes, collectively called CHASM (C Hepatitis Associated Systemic Manifestations) are present in a high proportion of infected persons. These include thyroid disease (Hashimoto’s thyroiditis, Graves disease, thyroid cancer), cardiovascular disease (atherosclerosis, carotid artery disease, coronary artery disease), renal disease (MPGN, glomerulosclerosis), eye disease (Mooren’s ulcers, sicca syndrome), skin disease (PCT, vasculitis, lichen planus), and lymphomas (NHL, splenic T-cell), and diabetes. Summary Mechanistic understanding of how HCV leads to CHASM processes could lead to development of new interventions. The role of early HCV treatment and cure may result in preventive strategies for a variety of complex disease states. PMID:26208812

  17. [A child with ictal fear as the primary epileptic manifestation].

    PubMed

    Inutsuka, Miki; Ogino, Tatsuya; Yoshinaga, Harumi; Ohtsuka, Yoko; Oka, Eiji

    2003-07-01

    We report a 4-year-old boy with ictal fear as his primary epileptic manifestation. Following an arrest of motion, the boy started to scream and struggle with an expression of horror on his face. Oral automatisms appeared around the end of the seizure. Complex visual and gustatory hallucinations and pain in the left leg were also observed. Ictal and interictal scalp EEGs revealed epileptic discharges in bilateral frontal regions. Ictal SPECT (99 mTc-HMPAO) showed hyperperfusion in right medial temporal area. These findings suggest that ictal fear associated with other ictal manifestations such as various hallucinations and oral automatisms resulted from rapid spread of epileptic discharges from frontal lobes to the right anterior temporal region.

  18. Ocular manifestations in the Hutchinson-Gilford progeria syndrome.

    PubMed

    Chandravanshi, Shivcharan L; Rawat, Ashok Kumar; Dwivedi, Prem Chand; Choudhary, Pankaj

    2011-01-01

    The Hutchinson-Gilford progeria (HGP) syndrome is an extremely rare genetic condition characterized by an appearance of accelerated aging in children. The word progeria is derived from the Greek word progeros meaning 'prematurely old'. It is caused by de novo dominant mutation in the LMNA gene (gene map locus 1q21.2) and characterized by growth retardation and accelerated degenerative changes of the skin, musculoskeletal and cardiovascular systems. The most common ocular manifestations are prominent eyes, loss of eyebrows and eyelashes, and lagophthalmos. In the present case some additional ocular features such as horizontal narrowing of palpebral fissure, superior sulcus deformity, upper lid retraction, upper lid lag in down gaze, poor pupillary dilatation, were noted. In this case report, a 15-year-old Indian boy with some additional ocular manifestations of the HGP syndrome is described.

  19. Staphylococcus aureus Infections: Epidemiology, Pathophysiology, Clinical Manifestations, and Management

    PubMed Central

    Davis, Joshua S.; Eichenberger, Emily; Holland, Thomas L.

    2015-01-01

    SUMMARY Staphylococcus aureus is a major human pathogen that causes a wide range of clinical infections. It is a leading cause of bacteremia and infective endocarditis as well as osteoarticular, skin and soft tissue, pleuropulmonary, and device-related infections. This review comprehensively covers the epidemiology, pathophysiology, clinical manifestations, and management of each of these clinical entities. The past 2 decades have witnessed two clear shifts in the epidemiology of S. aureus infections: first, a growing number of health care-associated infections, particularly seen in infective endocarditis and prosthetic device infections, and second, an epidemic of community-associated skin and soft tissue infections driven by strains with certain virulence factors and resistance to β-lactam antibiotics. In reviewing the literature to support management strategies for these clinical manifestations, we also highlight the paucity of high-quality evidence for many key clinical questions. PMID:26016486

  20. Cockayne syndrome without typical clinical manifestations including neurologic abnormalities.

    PubMed

    Miyauchi-Hashimoto, H; Akaeda, T; Maihara, T; Ikenaga, M; Horio, T

    1998-10-01

    Although patients with mild symptoms of atypical Cockayne syndrome (CS) have been described, there has not been a report of a patient with CS whose only clinical manifestation was cutaneous photosensitivity. Cells from patients with CS show UV sensitivity, reduced recovery of RNA synthesis, but normal UV-induced unscheduled DNA synthesis. On the other hand, the patients with UV-sensitive syndrome have only cutaneous photosensitivity and skin freckles, whereas those cells respond to UV radiation in a similar fashion to the CS cells. We describe a patient with CS who showed only photosensitivity without typical clinical manifestations of CS, but his cells showed UV sensitivity, reduced recovery of RNA synthesis, and normal unscheduled DNA synthesis after UV radiation similar to CS cells. Furthermore, the patient was assigned to complementation group B of CS on the basis of the results of complementation analysis. The present report suggests that CS has a wider spectrum than that considered previously. PMID:9777763

  1. Progressive outer retinal necrosis: manifestation of human immunodeficiency virus infection.

    PubMed

    Lo, Phey Feng; Lim, Rongxuan; Antonakis, Serafeim N; Almeida, Goncalo C

    2015-05-06

    We present the case of a 54-year-old man who developed progressive outer retinal necrosis (PORN) as an initial manifestation of HIV infection without any significant risk factors for infection with HIV. PORN is usually found as a manifestation of known AIDS late in the disease. Our patient presented with transient visual loss followed by decrease in visual acuity and facial rash. Subsequent investigation revealed anterior chamber tap positive for varicella zoster virus (VZV), as well as HIV positivity, with an initial CD4 count of 48 cells/µL. Systemic and intravitreal antivirals against VZV, and highly active antiretroviral therapy against HIV were started, which halted further progression of retinal necrosis. This case highlights the importance of suspecting PORN where there is a rapidly progressive retinitis, and also testing the patient for HIV, so appropriate treatment can be started.

  2. Oral manifestations of paracoccidioidomycosis. Report of 21 cases from Argentina.

    PubMed

    Godoy, H; Reichart, P A

    2003-01-01

    The present study describes 21 Argentinian patients living in the province of Corrientes, who had developed oral manifestations due to Paracoccidioides brasiliensis infection. Of these, 20 patients were men and one a woman. Patients were of an average age of 39 years (range 25 to 72 years). Approximately, 76.2% of the patients were farmers. Gingival lesions were observed in 76%. Also, the tongue (71%) and the lips (62%) were frequently affected. Cytological smears and histopathology showed the characteristic fungal cells with the characteristic granulomatous inflammatory reaction consisting of lymphocytes, epithelioid cells and giant cells of the Langhans type. All patients except one had detectable pulmonary involvement. Therapy consists of long-term administration of itraconazole. Oral manifestations of paracoccidioidomycosis are characteristic in their clinical presentation. Early diagnosis and adequate therapy may prevent extensive tissue destructions. Long-term follow-up is mandatory. PMID:14622391

  3. Extra-Renal Manifestations of Complement-Mediated Thrombotic Microangiopathies

    PubMed Central

    Hofer, Johannes; Rosales, Alejandra; Fischer, Caroline; Giner, Thomas

    2014-01-01

    Thrombotic microangiopathies (TMA) are rare but severe disorders, characterized by endothelial cell activation and thrombus formation leading to hemolytic anemia, thrombocytopenia, and organ failure. Complement over activation in combination with defects in its regulation is described in an increasing number of TMA and if primary for the disease denominated as atypical hemolytic-uremic syndrome. Although TMA predominantly affects the renal microvasculature, extra-renal manifestations are observed in 20% of patients including involvement of the central nerve system, cardiovascular system, lungs, skin, skeletal muscle, and gastrointestinal tract. Prompt diagnosis and treatment initiation are therefore crucial for the prognosis of disease acute phase and the long-term outcome. This review summarizes the available evidence on extra-renal TMA manifestations and discusses the role of acute and chronic complement activation by highlighting its complex interaction with inflammation, coagulation, and endothelial homeostasis. PMID:25250305

  4. Neurological counterparts of hyponatremia: pathological mechanisms and clinical manifestations.

    PubMed

    Podestà, Manuel Alfredo; Faravelli, Irene; Cucchiari, David; Reggiani, Francesco; Oldani, Silvia; Fedeli, Carlo; Graziani, Giorgio

    2015-04-01

    Hyponatremia, defined as a serum sodium concentration <135 mEq/L, represents the most frequent electrolyte disorder in older hospitalized patients. Early recognition of hyponatremia is mandatory, since it represents an independent risk factor that increases hospital mortality by 40 %. Delayed correction of hyponatremia may worsen brain edema, resulting in different degrees of neural damage. However, an overly rapid correction of serum sodium levels can lead to osmotic demyelination syndrome (ODS), a dreadful neurological picture. In recent years, hyponatremia and ODS have received growing attention both in terms of clinical management and pathophysiology, leading to the discovery of new drugs and treatment algorithms. In this review, we recapitulate the pathogenetic background, clinical manifestations, and treatment guidelines of hyponatremia, focusing on the neurological alterations. Neurological symptoms may be neglected when they manifest as early signs of mild hyponatremia, while brain damage can irremediably affect patients' conditions in the context of ODS.

  5. Current perspectives on ophthalmic manifestations of childhood rheumatic diseases.

    PubMed

    Palejwala, Neal V; Yeh, Steven; Angeles-Han, Sheila T

    2013-07-01

    Inflammatory eye diseases are an important manifestation of many pediatric rheumatologic conditions. Early screening and diagnosis are imperative as these illnesses can not only result in significant visual morbidity but are also an indicator of systemic inflammation. Time to presentation of ocular inflammation varies significantly and can range from many years prior to the onset of systemic symptoms to well after the diagnosis of the rheumatologic disorder. Due to this variability in presentation, careful monitoring by an ophthalmologist is vital to preventing ocular complications and preserving vision. Both local and systemic immunosuppressive medications have been effective in the management of ocular disease. In this review, we will focus on the known ophthalmologic manifestations of common pediatric rheumatologic diseases and discuss recent advances in therapeutic considerations for these conditions. PMID:23686303

  6. Panniculitis: an unusual presenting manifestation of rheumatoid arthritis.

    PubMed

    Fragoulis, George E; Stamopoulos, Paraskevas; Barbatis, Calypso; Tzioufas, Athanasios G

    2016-01-01

    Rheumatoid arthritis (RA) is a systemic inflammatory disease affecting primarily the joints but also other organs including skin. Panniculitis is an extremely rare manifestation of the disease manifesting mainly as reddish, ulcerative painful nodules and papules, usually in the legs. Histopathologically, it is characterised by liponecrobiosis, granulocytic and histiocytic infiltrates and vasculopathy. Herein, we describe a middle-aged woman with past medical history of hypertension and diabetes mellitus, and unremarkable family history, who presented with symmetrical polyarthritis, low grade fever and painful subcutaneous nodules in the abdomen. Her laboratory tests showed high acute phase reactants, positive rheumatoid factor and anti-Ro autoantibodies and negative anti-CCP. Surgical resection and histological examination of the nodules revealed neutrophilic lobular panniculitis associated with RA. She was treated with low doses of glucocorticosteroids and methotrexate. The latter was substituted with leflunomide due to toxicity. The patient had significant clinical and laboratory improvement.

  7. Unusual manifestations in dengue outbreak 2009, Delhi, India.

    PubMed

    Jhamb, Rajat; Kumar, Ashok; Ranga, G S; Rathi, Nitin

    2010-12-01

    Epidemics of Dengue fever (DF) and Dengue hemorrhagic fever (DHF) are common in Southeast Asia. DF is defined & classified according to WHO criteria. Variable clinical manifestations of DF & DHF have been described in earlier studies. But some patients present with unusual clinical features and clinical profile not classifiable according to the present WHO criteria. Some of these complications if not recognized early and treated properly can even prove fatal. So this study was done to describe various clinical features in Dengue fever with special emphasis on unusual manifestations. This study was conducted at University College of Medical Sciences and associated Guru Teg Bahadur hospital; a tertiary care hospital, located in East Delhi in India. It was a retrospective study of 76 patients of probable DF; including 4 cases of DHF (according to WHO classification); 60 males & 16 females above 12 years of age admitted in medical wards of Guru Teg Bahadur hospital in an outbreak of DF which occurred during September-December 2009. The data obtained was analyzed to see clinical and laboratory profile of DF/DHF with special emphasis on unusual manifestations. The mean age of the patients was 28 +/- 9.6 years. Fever was present in all the cases with an average duration of fever being 5.47 +/- 2.2 days with body ache, (84.2%), vomiting (61.8%), abdominal pain (51.3%) and headache (19.7%) being the other presenting complaints. Hemorrhagic manifestations in the form of gum bleeding and epistaxis (35.5%), positive tourniquet test (27.6%); skin rashes (15.8%), melena (15.8%) and hematemesis (5.26%) were also present. In our study a fair no of patients presented with unusual symptoms like pain in abdomen 39 (51.3%), nausea 32 (42.1%), & vomiting 47 (61.8%), which is higher than that reported previous outbreak. Of the 39 patients who presented with abdominal pain; ultrasonography of abdomen was done in 25 patients. Fifteen (38%) of these were found to have acalculous

  8. Langerhans Histiocytosis in a Child – Diagnosed by Oral Manifestations

    PubMed Central

    Golai, Shruti; Patil, Sandya Devi; Kakanur, Madhu; Paul, Santosh

    2015-01-01

    Langerhans Histiocytosis (LCH) is a rare reactive and proliferative disease of histiocytes with unknown etiology, characterized by excessive proliferation of histiocytes called Langerhans cells. It occurs mainly in children but occurrence in adults has also been reported. It manifests as punched out lesions in the skull, maxilla, mandible, sternum and other flat bones and causes rapid resorption of the alveolar bone leading to floating teeth appearance in the radiographs. This disease manifests initially in the oral cavity in most of the cases and can be diagnosed by careful clinical and radiological examination. Here, we present a case of LCH in a child which was diagnosed by a swelling in the mandibular region. PMID:26023652

  9. [Clinical manifestations of hematological non-neoplastic diseases in Dentistry].

    PubMed

    Bascones-Martínez, Antonio; Muñoz-Corcuera, Marta; Bascones-Ilundain, Cristina

    2012-06-01

    Systemic disease can cause clinical manifestations in the oral and maxillofacial area, which is important to recognize because it could be the first symptom of an undiagnosed illness. There are different oral signs that could suggest the clinician a blood disorder, such as pallor, petechiae, ecchymosis, ulcerations, gingival hypertrophy or spontaneous gingival bleeding. In addition, blood disorders will determine the dental management of these patients and the protocol for limiting possible complications that may arise due to the treatment itself. This paper reviews the oral manifestations and dental management of non-neoplastic alterations of red cells, white cells and hemostasis, with emphasis on two-way relationship that must exist between the dentist and the patient's hematologist for making a treatment plan.

  10. The MANIFEST fibre positioning system for the Giant Magellan Telescope

    NASA Astrophysics Data System (ADS)

    Lawrence, Jon S.; Brown, David M.; Brzeski, Jurek; Case, Scott; Colless, Matthew; Farrell, Tony; Gers, Luke; Gilbert, James; Goodwin, Michael; Jacoby, George; Hopkins, Andrew M.; Ireland, Michael; Kuehn, Kyler; Lorente, Nuria P. F.; Miziarski, Stan; Muller, Rolf; Nichani, Vijay; Rakman, Azizi; Richards, Samuel; Saunders, Will; Staszak, Nick F.; Tims, Julia; Vuong, Minh; Waller, Lew

    2014-08-01

    MANIFEST is a fibre feed system for the Giant Magellan Telescope that, coupled to the seeing-limited instruments GMACS and G-CLEF, offers qualitative and quantitative gains over each instrument's native capabilities in terms of multiplex, field of view, and resolution. The MANIFEST instrument concept is based on a system of semi-autonomous probes called "Starbugs" that hold and position hundreds of optical fibre IFUs under a glass field plate placed at the GMT Cassegrain focal plane. The Starbug probes feature co-axial piezoceramic tubes that, via the application of appropriate AC waveforms, contract or bend, providing a discrete stepping motion. Simultaneous positioning of all Starbugs is achieved via a closed-loop metrology system.

  11. [Skin manifestations in lupus erythematosus: clinical aspects and therapy].

    PubMed

    Kuhn, A; Ruland, V; Bonsmann, G

    2011-04-01

    Lupus erythematosus (LE) is an inflammatory autoimmune disorder, which is characterized by clinically heterogeneous manifestations of different organs. In systemic LE (SLE) the skin, the musculoskeletal system, the kidneys, the cardiovascular and central nervous systems can be involved. The skin lesions can be divided into LE-specific and LE-non-specific manifestations, the former represent the subtypes of cutaneous LE (CLE). The diagnosis is confirmed by clinical, histopathological, immunoserological and genetic features. The treatment is similar for the different subtypes of CLE; however, the therapeutic regimen should be individually defined in each patient. Antimalarials are still the first-line systemic therapy and in addition to sunscreens, glucocorticosteroids and calcineurin inhibitors have an important impact as topical agents in this disease.

  12. Interstitial granulomatous dermatitis: rare cutaneous manifestation of rheumatoid arthritis*

    PubMed Central

    Veronez, Isis Suga; Dantas, Fernando Luiz; Valente, Neusa Yuriko; Kakizaki, Priscila; Yasuda, Thaís Helena; Cunha, Thaís do Amaral

    2015-01-01

    Besides being an uncommon clinicopathological entity, interstitial granulomatous dermatitis, also described as interstitial granulomatous dermatitis with arthritis (IGDA), has shown a wide spectrum of clinical manifestations, such as linear and erythematous lesions, papules, plaques and nodules. Histological features include dense dermal histiocytic infiltrate, usually in a palisade configuration, and scattered neutrophils and eosinophils. We describe a middle aged woman with rheumatoid arthritis of difficult management and cutaneous lesions compatible with IGDA. PMID:26131871

  13. Eczema as the first manifestation of a lung cancer.

    PubMed

    Meng, Xiangjiao; Wang, Renben; Huang, Zhaoqin; Yu, Jinming

    2015-01-01

    Lung cancer combined with eczema is a rare disease. We report a case of 58-year-old man with eczema as the first manifestation of a lung cancer. Skin examination revealed diffuse erythema, dander, itchy rash, and scratch. Chest contrast-enhanced computed tomography showed a heterogeneously enhanced irregular mass in the right lung. Punch biopsy of the tumor confirms squamous cell lung cancer. Eczema vanished nearly completely after one cycle of chemotherapy.

  14. Hypokalemic rhabdomyolysis: a rare manifestation of primary aldosteronism.

    PubMed

    Zavatto, A; Concistrè, A; Marinelli, C; Zingaretti, V; Umbro, I; Fiacco, F; Tinti, F; Petramala, L; Mitterhofer, A P; Letizia, C

    2015-10-01

    Rhabdomyolysis is a rare presentation of hypokalemia, although muscle weakness is a well-known manifestation of hypokalemia. Primary aldosteronism is characterized by hypertension, suppressed plasma renin activity, increased aldosterone excretion and hypokalemia with metabolic alkalosis. Rhabdomyolysis is not common in primary aldosteronism. We present here a 40-year-old woman presenting with rhabdomyolysis accompanied by severe hypokalemia as heralding symptom of primary aldosteronism.

  15. Pulmonary Embolism as the First Manifestation of Multiple Myeloma

    PubMed Central

    Vallianou, N.; Lazarou, V.; Tzangarakis, J.; Barounis, R.; Sioula, E.

    2013-01-01

    Multiple myeloma is considered a hypercoagulable state due to several mechanisms such as the increased IL-6 and immunoglobulins production, the defective fibrinolytic mechanism, and the acquired resistance to activated protein C that are involved in the pathogenesis and clinical futures of the disease. We describe a case of a female patient who presented to the hospital with pulmonary embolism as the first manifestation of the hypercoagulability of multiple myeloma. PMID:24151508

  16. Radar manifestations of ship wakes in algae bloom zones

    NASA Astrophysics Data System (ADS)

    Mityagina, Marina I.; Lavrova, Olga Yu.

    2014-10-01

    Radar manifestations of ship wakes in zones of phytoplankton bloom are discussed. It is shown that these signatures can be regarded as indicators of biogenic activity. The main data are satellite radar images. Satellite visible (VIS) and infrared (IR) satellite data are also analyzed. The large amount of the available data allowed us to make some generalizations and obtain statistically reliable results concerning spatial and temporal variability of certain type of ship wake manifestations in synthetic aperture radar (SAR) images of the sea surface. Traditional classification of surface ship wakes manifestations in satellite SAR images specifies distinct features such as a dark trailing centreline region (turbulent wake), narrow V-wakes aligned at some angle to the ship's path (the Kelvin wake), and, sometimes, internal wave wakes generated under conditions of shallow stratification. Their characteristic lengths are reported to be up to tens of kilometers and they can last from tens of minutes up to one hour. Instances of radar signatures of the ship wakes dissimilar to the previously described were detected in radar images obtained in the course of a satellite monitoring campaign of the central and south-eastern Baltic. These ship wakes can be seen in satellite radar images as long bright strips of enhanced backscatter with characteristic length of up to several hundred kilometres lasting more than 5 hours. A hypothesis is put forward of the coherence of this type of ship wakes detected in sea surface radar imagery and areas of intensive biogenic activity under conditions of low near-surface winds. Statistics on their seasonal, spatial and year-to-year distribution are drawn. These results are compared with temporal and spatial variations in chlorophyll a concentration and intensity of phytoplankton bloom in the area of interest. Chlorophyll a concentration maps derived from satellite data are used, as well as those based on in situ measurements. The relation

  17. Rare dental manifestation in Simpson-Golabi-Behmel syndrome.

    PubMed

    Parashar, Pallavi; Preston, Sally; Brada, Brian; Borris, Thomas; Potter, Brad

    2016-01-01

    Simpson-Golabi-Behmel syndrome (SGBS) is a rare X-linked recessive overgrowth disorder with prominent craniofacial manifestations. Macrodontia is also an uncommon dental anomaly that can be an isolated finding and has been associated with numerous systemic conditions and syndromes. This case report describes this previously unreported dental anomaly, macrodontia, in a patient with SGBS, which may broaden the phenotype of this syndrome. A brief review of the literature on orofacial findings associated with SGBS is also presented.

  18. Sickle cell anemia oral manifestations in a Venezuelan population.

    PubMed

    Saint Clair de Velasquez, Y; Rivera, H

    1997-01-01

    Sickle cell anemia (SCA) is a well known hemoglobinopathy which results from a substitution of amino acids in the polypeptidic chain. SCA was considered endemic in certain areas of the world. It has been recognized now that it may have a wide geographic distribution. Few studies have dealt with dental manifestations or complications of SCA (Cox and Soni, 1984). Nevertheless none of them have showed epidemiological data for a large series of oral manifestations. To date, no epidemiological data of our country is available in the literature. The aim of this study was to determine the oral manifestations of SCA in a Venezuelan population. Seventeen patients affected were examined at the University Hospital and the Dental Clinic. Age ranged between 1 1/2-48 years. Each patient was haematologically diagnosed by hemoglobin electrophoresis and only homozygous individuals were selected. Each patient was analyzed according to general clinical history, as well as, dental history; clinical and radiological examination using periapical, panorex and bite-wings radiographs. Our results showed that the most affected group was between 20 to 30 years (41.18%). According to sex, females were more affected than males (64.71%). The most common phenotype was mestizo (47.31%). The most frequent type of hemoglobinopathy was Hg-SS and Hg SS-F. The most common soft tissue oral manifestation was buccal mucosa pallor in 77.05%. In addition, the hard tissue findings involved enlarged medullary spaces (70.58%). Cicatritial infarcts were present in 77.05% of cases and the step-ladder effect was demonstrated in 82.35% of cases. Our observations could be due to genetic, environmental, nutritional and geographical factors. PMID:11885236

  19. Skin manifestations of acromegaly - a study of 34 cases.

    PubMed

    Arya, K R; Krishna, K; Chadda, M

    1997-01-01

    The common dermatological manifestations seen in 34 cases of acromegaly were changes in facial appearance, enlargement of hands and feet, intolerance to heat and sweating, carpal tunnel syndrome, hirsutism, acrochordons and acanthosis nigricans. The mean estimated age of onset was 32.8 years in males and 31.7 years in females, while the mean age at the time of diagnosis was 38.6 years and 36.1 years for males and females respectively, with a slight male preponderance noted.

  20. Apparent Lenz microphthalmia syndrome: a patient with unusual manifestations.

    PubMed

    Okumus, N; Zenciroglu, A; Demirel, N; Bas, A Y; Ceylaner, S

    2008-01-01

    Lenz microphthalmia syndrome was first described by Lenz et al. in 1955 (9). The cardinal features of the syndrome are microphthalmia or anophthalmia, microcephaly, mental retardation, external ear, digital, cardiac, skeletal, dental and genitourinary anomalies. Here we present a case of Lenz microphthalmia syndrome that shows the typical characteristics and, additionally, macrophallus, a broad chest with widely spaced nipples, wide gap between first and second toes, which are unusual manifestations in Lenz Microphthalmia Syndrome.

  1. Silent intravascular lymphoma initially manifesting as a unilateral adrenal incidentaloma.

    PubMed

    Takahashi, Yoshiko; Iida, Keiji; Hino, Yasuhisa; Ohara, Takeshi; Kurahashi, Toshifumi; Tashiro, Takashi; Chihara, Kazuo

    2012-01-01

    Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of malignant lymphoma. Although the involvement of adrenal glands in IVLBCL is often observed, primary adrenal IVLBCL is rare. Most reported cases of adrenal IVLBCL showed bilateral lesions resulting in rapidly progressive adrenal failure and poor prognosis. Here, we report a case of slowly progressive primary adrenal IVLBCL manifesting initially with unilateral adrenal incidentaloma. This case is a silent IVLBCL and shows that the enlargement of both adrenal glands can be followed.

  2. Arrhythmia as a cardiac manifestation in MELAS syndrome.

    PubMed

    Thomas, Tamara; Craigen, William J; Moore, Ryan; Czosek, Richard; Jefferies, John L

    2015-09-01

    A 44-year-old female with a diagnosis of mitochondrial myopathy, encephalopathy and stroke-like episodes (MELAS) syndrome had progressive left ventricular hypertrophy (LVH) on echocardiogram. A Holter monitor demonstrated episodes of non-sustained atrial tachycardia, a finding not been previously described in this population. This unique case of MELAS syndrome demonstrates the known associated cardiac manifestation of LVH and the new finding of atrial tachycardia which may represent the potential for subclinical arrhythmia in this population.

  3. Ocular manifestations of monoclonal copper-binding immunoglobulin.

    PubMed

    Shah, Sejal; Espana, Edgar M; Margo, Curtis E

    2014-01-01

    The dense accumulation of copper in Descemet membrane and lens capsule is the characteristic manifestation of a circulating monoclonal antibody with strong affinity for copper. The overproduction of this monoclonal immunoglobulin may be associated with either multiple myeloma or a benign monoclonal gammopathy. Despite prolonged exposure to elevated serum copper, no other tissues in the body are adversely affected by this redox metal. We describe the clinical and pathological findings in a 46-year-old woman with this disorder.

  4. Osseous manifestations of elbow stress associated with sports activities.

    PubMed

    Gore, R M; Rogers, L F; Bowerman, J; Suker, J; Compere, C L

    1980-05-01

    The radiographic manifestations of musculoskeletal stress at the elbow associated with racket and throwing sports are reported in 29 symptomatic amateur, juvenile, and professional athletes. Five major categories of stress are identified: diffuse, humeral shaft, medial tension, lateral compression, and extension stress. The osseous changes produced by these stresses include bony hypertrophy, loose bodies, traction spur formation, osteochondral and humeral shaft fractures in the adult, and epiphyseal and apophyseal hypermaturity or avulsion in the youngster.

  5. Osteoskeletal manifestations of scurvy: MRI and ultrasound findings.

    PubMed

    Polat, Ahmet Veysel; Bekci, Tumay; Say, Ferhat; Bolukbas, Emrah; Selcuk, Mustafa Bekir

    2015-08-01

    Scurvy has become very rare in the modern world. The incidence of scurvy in the pediatric population is extremely low. In the pediatric population, musculoskeletal manifestations are more common and multiple subperiosteal hematomas are an important indicator for the diagnosis of scurvy. Although magnetic resonance imaging findings of scurvy are well described in the literature, to our knowledge, ultrasound findings have not yet been described. In this article, we report a case of scurvy with associated magnetic resonance imaging and ultrasound findings.

  6. Extra-articular manifestations of rheumatoid arthritis: An update.

    PubMed

    Prete, Marcella; Racanelli, Vito; Digiglio, Liboria; Vacca, Angelo; Dammacco, Franco; Perosa, Federico

    2011-12-01

    Rheumatoid arthritis (RA) is an immune-mediated disease involving chronic low-grade inflammation that may progressively lead to joint destruction, deformity, disability and even death. Despite its predominant osteoarticular and periarticular manifestations, RA is a systemic disease often associated with cutaneous and organ-specific extra-articular manifestations (EAM). Despite the fact that EAM have been studied in numerous RA cohorts, there is no uniformity in their definition or classification. This paper reviews current knowledge about EAM in terms of frequency, clinical aspects and current therapeutic approaches. In an initial attempt at a classification, we separated EAM from RA co-morbidities and from general, constitutional manifestations of systemic inflammation. Moreover, we distinguished EAM into cutaneous and visceral forms, both severe and not severe. In aggregated data from 12 large RA cohorts, patients with EAM, especially the severe forms, were found to have greater co-morbidity and mortality than patients without EAM. Understanding the complexity of EAM and their management remains a challenge for clinicians, especially since the effectiveness of drug therapy on EAM has not been systematically evaluated in randomized clinical trials. PMID:21939785

  7. Pulmonary manifestations of Sjögren's syndrome.

    PubMed

    Flament, Thomas; Bigot, Adrien; Chaigne, Benjamin; Henique, Helene; Diot, Elisabeth; Marchand-Adam, Sylvain

    2016-06-01

    In 9-20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD) and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients.

  8. Personality disorders among alcohol-dependent patients manifesting or not manifesting cocaine abuse: a comparative pilot study.

    PubMed

    Echeburúa, Enrique; De Medina, Ricardo Bravo; Aizpiri, Javier

    2009-01-01

    This study assessed personality disorders (PDs) in 158 alcohol-dependent outpatients (62 manifesting cocaine abuse and 96 without cocaine abuse) with the International Personality Disorders Examination interview between 2003 and 2006. Thirty-nine alcohol-dependent/cocaine abusers (62.9% of this group) and 51 only alcohol-dependent patients (53.1% of this group) manifested at least one PD. There were no statistically significant differences between groups in the overall prevalence rate of PDs. The most prevalent PDs, among the alcohol-dependent/cocaine abusers, were antisocial (21%), narcissistic (14.5%), and borderline (11.3%) PDs. The most frequently diagnosed PDs among the only alcohol-dependent patients were obsessive-compulsive (20.8%), paranoid (10.4%), and dependent (9.4%) PDs. There were significant differences between the groups. The study limitations are discussed.

  9. Congenital retrosternal hernias of Morgagni: Manifestation and treatment in children

    PubMed Central

    Slepov, Oleksii; Kurinnyi, Sergii; Ponomarenko, Oleksii; Migur, Mikhailo

    2016-01-01

    Background: Due to scarcity of congenital diaphragmatic hearnias of Morgagni (CDHM), non-specific clinical presentation in the pediatric age group, we aimed to investigate the incidence, clinical manifestations, anatomical characteristics, and develop diagnostic algorithm and treatment of CDHM in children. Materials and Methods: The patients’ records of children with CDHM treated in our hospital during past 20 years were retrospectively reviewed for the age at diagnosis, gender, clinical findings, anatomical features, operative details and outcome. Results: Since 1995 to 2014 we observed 6 (3 boys, 3 girls) patients with CDHM, that comprise 3.2% of all congenital diaphragmatic hernia cases (n = 185). Age at diagnosis varied from 3 mo. to 10y.o. Failure to thrive was main symptom in 4 patients, followed by recurrent respiratory infections (n = 3), dyspnea (n = 3), and gastrointestinal manifestations: constipation (n = 2), abdominal pain (n = 1). Work-up consisted of plain X-ray for all (n = 6), upper GI (n = 3), barium enema (n = 2), sonography (n = 6) and CT (n = 2). Abdominal approach used in 5 patients, and thoracotomy in one. Herniated contents were: liver lobes (n = 4), transverse colon (n = 3) and greater omentum (n = 1). 5 had right-sided lesion, 1- left-sided. Defect repaired using local tissues. Post-operative course was uneventful; all patients appeared well during follow-up. Conclusion: CDHM is very uncommon anomaly, very occasionally diagnosed at the early age. Failure to thrive and recurrent respiratory infections are most frequent clinical manifestations. In suspected CDHM we advocate the following work-up: plain chest and abdominal X-ray, contrast study (upper GI series or barium enema), ultrasonographic screen and CT scan. Surgical repair via abdominal approach, using local tissues and hernia sac removal is preferred. PMID:27251653

  10. Ionospheric manifestations of earthquakes and tsunamis in a dynamic atmosphere

    NASA Astrophysics Data System (ADS)

    Godin, Oleg A.; Zabotin, Nikolay A.; Zabotina, Liudmila

    2015-04-01

    Observations of the ionosphere provide a new, promising modality for characterizing large-scale physical processes that occur on land and in the ocean. There is a large and rapidly growing body of evidence that a number of natural hazards, including large earthquakes, strong tsunamis, and powerful tornadoes, have pronounced ionospheric manifestations, which are reliably detected by ground-based and satellite-borne instruments. As the focus shifts from detecting the ionospheric features associated with the natural hazards to characterizing the hazards for the purposes of improving early warning systems and contributing to disaster recovery, it becomes imperative to relate quantitatively characteristics of the observed ionospheric disturbances and the underlying natural hazard. The relation between perturbations at the ground level and their ionospheric manifestations is strongly affected by parameters of the intervening atmosphere. In this paper, we employ the ray theory to model propagation of acoustic-gravity waves in three-dimensionally inhomogeneous atmosphere. Huygens' wavefront-tracing and Hamiltonian ray-tracing algorithms are used to simulate propagation of body waves from an earthquake hypocenter through the earth's crust and ocean to the upper atmosphere. We quantify the influence of temperature stratification and winds, including their seasonal variability, and air viscosity and thermal conductivity on the geometry and amplitude of ionospheric disturbances that are generated by seismic surface waves and tsunamis. Modeling results are verified by comparing observations of the velocity fluctuations at altitudes of 150-160 km by a coastal Dynasonde HF radar system with theoretical predictions of ionospheric manifestations of background infragravity waves in the ocean. Dynasonde radar systems are shown to be a promising means for monitoring acoustic-gravity wave activity and observing ionospheric perturbations due to earthquakes and tsunamis. We will discuss

  11. Neurologic Manifestations of Enterovirus 71 Infection in Korea.

    PubMed

    Lee, Kyung Yeon; Lee, Myoung Sook; Kim, Dong Bin

    2016-04-01

    Enterovirus 71 frequently involves the central nervous system and may present with a variety of neurologic manifestations. Here, we aimed to describe the clinical features, magnetic resonance imaging (MRI) findings, and cerebrospinal fluid (CSF) profiles of patients presenting with neurologic complications of enterovirus 71 infection. We retrospectively reviewed the records of 31 pediatric patients hospitalized with acute neurologic manifestations accompanied by confirmed enterovirus 71 infection at Ulsan University Hospital between 2010 and 2014. The patients' mean age was 2.9 ± 5.5 years (range, 18 days to 12 years), and 80.6% of patients were less than 4 years old. Based on their clinical features, the patients were classified into 4 clinical groups: brainstem encephalitis (n = 21), meningitis (n = 7), encephalitis (n = 2), and acute flaccid paralysis (n = 1). The common neurologic symptoms included myoclonus (58.1%), lethargy (54.8%), irritability (54.8%), vomiting (48.4%), ataxia (38.7%), and tremor (35.5%). Twenty-five patients underwent an MRI scan; of these, 14 (56.0%) revealed the characteristic increased T2 signal intensity in the posterior region of the brainstem and bilateral cerebellar dentate nuclei. Twenty-six of 30 patients (86.7%) showed CSF pleocytosis. Thirty patients (96.8%) recovered completely without any neurologic deficits; one patient (3.2%) died due to pulmonary hemorrhage and shock. In the present study, brainstem encephalitis was the most common neurologic manifestation of enterovirus 71 infection. The characteristic clinical symptoms such as myoclonus, ataxia, and tremor in conjunction with CSF pleocytosis and brainstem lesions on MR images are pathognomonic for diagnosis of neurologic involvement by enterovirus 71 infection. PMID:27051240

  12. Neurologic Manifestations of Enterovirus 71 Infection in Korea

    PubMed Central

    2016-01-01

    Enterovirus 71 frequently involves the central nervous system and may present with a variety of neurologic manifestations. Here, we aimed to describe the clinical features, magnetic resonance imaging (MRI) findings, and cerebrospinal fluid (CSF) profiles of patients presenting with neurologic complications of enterovirus 71 infection. We retrospectively reviewed the records of 31 pediatric patients hospitalized with acute neurologic manifestations accompanied by confirmed enterovirus 71 infection at Ulsan University Hospital between 2010 and 2014. The patients’ mean age was 2.9 ± 5.5 years (range, 18 days to 12 years), and 80.6% of patients were less than 4 years old. Based on their clinical features, the patients were classified into 4 clinical groups: brainstem encephalitis (n = 21), meningitis (n = 7), encephalitis (n = 2), and acute flaccid paralysis (n = 1). The common neurologic symptoms included myoclonus (58.1%), lethargy (54.8%), irritability (54.8%), vomiting (48.4%), ataxia (38.7%), and tremor (35.5%). Twenty-five patients underwent an MRI scan; of these, 14 (56.0%) revealed the characteristic increased T2 signal intensity in the posterior region of the brainstem and bilateral cerebellar dentate nuclei. Twenty-six of 30 patients (86.7%) showed CSF pleocytosis. Thirty patients (96.8%) recovered completely without any neurologic deficits; one patient (3.2%) died due to pulmonary hemorrhage and shock. In the present study, brainstem encephalitis was the most common neurologic manifestation of enterovirus 71 infection. The characteristic clinical symptoms such as myoclonus, ataxia, and tremor in conjunction with CSF pleocytosis and brainstem lesions on MR images are pathognomonic for diagnosis of neurologic involvement by enterovirus 71 infection. PMID:27051240

  13. A study of neuropsychiatric manifestations in patients of neurocysticercosis

    PubMed Central

    Srivastava, Smita; Chadda, Rakesh Kumar; Bala, Kiran; Majumdar, Pradipta

    2013-01-01

    Background: Neurocysticercosis (NCC) is an endemic parasitic infection of Asia, Africa, Latin America, and central Europe. Neuropsychiatric manifestations of the illness include epilepsy and behavioral disturbances. There is a dearth of systematic studies on psychiatric manifestations of NCC from various Asian counties. The present study assessed the prevalence of various psychiatric disorders in a cohort of patients with NCC attending a neurological service. Materials and Methods: Detailed psychiatric assessment was carried out on 50 patients of NCC with epilepsy and 50 patients of epilepsy without any evidence of NCC. Comprehensive Psychopathological Rating Scale was used to elicit the symptoms. Cognitive functions were assessed using Mini Mental Status Examination. Psychiatric diagnoses were made as per International Classification of Diseases, 10th edition (ICD-10). Results: Sixty eight percent of the patients with NCC suffered from a psychiatric disorder, as compared to 44% of those without NCC (P=0.02). Major depression and mixed anxiety depression were the two most common diagnoses. None of the patients was to found to suffer from a psychotic disorder. The most frequent site of brain lesion of NCC was the parietal lobe, followed by frontal lobes and disseminated lesions. Left sided lesions were associated with greater psychiatric morbidity. Focal seizures with or without secondary generalizations were present more frequently in patients with NCC whereas primary generalized seizures were more common in patients with idiopathic epilepsy (P=0.05). Conclusion: Psychiatric manifestations are more common in patients of epilepsy with NCC than those without NCC. The treating clinician need to be vigilant about the phenomenon. PMID:24082247

  14. Urban legends series: oral manifestations of HIV infection.

    PubMed

    Patton, L L; Ramirez-Amador, V; Anaya-Saavedra, G; Nittayananta, W; Carrozzo, M; Ranganathan, K

    2013-09-01

    Human immunodeficiency virus-related oral lesions (HIV-OLs), such as oral candidiasis (OC) and oral hairy leukoplakia (OHL), have been recognized as indicators of immune suppression since the beginning of the global HIV epidemic. The diagnosis and management of HIV disease and spectrum of opportunistic infection has changed over the past 30 years as our understanding of the infection has evolved. We investigated the following controversial topics: (i) Are oral manifestations of HIV still relevant after the introduction of highly active antiretroviral therapy (HAART)? (ii) Can we nowadays still diagnose HIV infection through oral lesions? (iii) Is the actual classification of oral manifestations of HIV adequate or does it need to be reviewed and updated? (iv) Is there any novelty in the treatment of oral manifestations of HIV infection? Results from extensive literature review suggested the following: (i) While HAART has resulted in significant reductions in HIV-OLs, many are still seen in patients with HIV infection, with OC remaining the most common lesion. While the relationship between oral warts and the immune reconstitution inflammatory syndrome is less clear, the malignant potential of oral human papillomavirus infection is gaining increasing attention. (ii) Effective antiretroviral therapy has transformed HIV from a fatal illness to a chronic manageable condition and as a result expanded screening policies for HIV are being advocated both in developed and in developing countries. Affordable, reliable, and easy-to-use diagnostic techniques have been recently introduced likely restricting the importance of HIV-OLs in diagnosis. (iii) The 1993 EC-Clearinghouse classification of HIV-OLs is still globally used despite controversy on the relevance of periodontal diseases today. HIV-OL case definitions were updated in 2009 to facilitate the accuracy of HIV-OL diagnoses by non-dental healthcare workers in large-scale epidemiologic studies and clinical trials. (iv

  15. The Spectrum of Parkinsonian Manifestations Associated With Glucocerebrosidase Mutations

    PubMed Central

    Goker-Alpan, Ozlem; Lopez, Grisel; Vithayathil, Joseph; Davis, Joie; Hallett, Mark; Sidransky, Ellen

    2008-01-01

    Background Mutations in the glucocerebrosidase gene (GBA) result in Gaucher disease and can be associated with a phenotype characterized by adult-onset progressive neurologic deterioration and parkinsonism. Objective To define the clinical and neurologic spectrum of parkinsonian manifestations associated with GBA mutations. Design, Setting, and Patients A prospective case series of 10 patients (7 men and 3 women) with parkinsonism and GBA mutations evaluated at the National Institutes of Health Clinical Center. Main Outcome Measures The GBA genotypes were identified by means of DNA sequencing. Tests evaluating neurologic, motor, cognitive, ocular, and olfactory functions were performed and the results were analyzed by a single team. Results Genotyping identified GBA mutations N370S, L444P, and c.84dupG and recombinant alleles. The mean age at onset of parkinsonian manifestations was 49 years (range, 39–65 years), disease duration was 7.8 years (range, 1.2–16.0 years), and Unified Parkinson Disease Rating Scale part III score was 26.3 (range, 13–38). Half of the patients reported cognitive changes later in the disease course. Six patients were diagnosed as having Parkinson disease, 3 as having Lewy body dementia, and 1 as having a “Parkinson plus” syndrome. The most frequent nonmotor finding was olfactory dysfunction. Atypical manifestations included myoclonus, electroencephalographic abnormalities, and seizures. Conclusions In the homozygous and heterozygous states, GBA mutations are associated with a spectrum of parkinsonian phenotypes ranging from Parkinson disease, mostly of the akinetic type, to a less common phenotype characteristic of Lewy body dementia. PMID:18852351

  16. Dandy-Walker Malformation Presenting with Psychological Manifestations.

    PubMed

    Rohanachandra, Yasodha Maheshi; Dahanayake, Dulangi Maneksha Amerasinghe; Wijetunge, Swarna

    2016-01-01

    Dandy-Walker malformation, which is a congenital malformation of the cerebellum, is documented in literature to be associated with psychotic symptoms, obsessive compulsive symptoms, mood symptoms, hyperactivity, and impulsive behavior. The pathogenesis of psychiatric symptoms in Dandy-Walker malformation is thought to be due to disruption of the corticocerebellar tracts, resulting in what is known as cerebellar cognitive affective syndrome. We present a case of Dandy-Walker malformation presenting with psychiatric symptoms. This case highlights the necessity to be aware of psychiatric manifestations of cerebellar disease as it has an impact on the diagnosis and treatment. PMID:27493822

  17. [Juvenil idiopathic arthritis. Part 1: diagnosis, pathogenesis and clinical manifestations].

    PubMed

    Espada, Graciela

    2009-10-01

    Juvenile idiopathic arthritis is not a single disease and constitutes an heterogeneous group of illnesses or inflammatory disorders. This new nomenclature encompasses different disease categories, each of which has different presentation, clinical signs, symptoms, and outcome. The cause of the disease is still unknown but both environmental and genetic factors seem to be related to its pathogenesis. Is the most common chronic rheumatic disease in children and an important cause of short-term and long-term disability. In this article, clinical manifestation, new classification and approach to diagnosis are reviewed.

  18. An alternative NMSSM phenomenology with manifest perturbative unification

    SciTech Connect

    Hall, Lawrence; Barbieri, Riccardo; Pappadopulo, Duccio; Rychkov, Vyacheslav S.; Hall, Lawrence J.; Papaioannou, Anastasios Y.

    2007-12-18

    Can supersymmetric models with a moderate stop mass be made consistent with the negative Higgs boson searches at LEP, while keeping perturbative unification manifest? The NMSSM achieves this rather easily, but only if extra matter multiplets filling complete SU(5) representations are present at intermediate energies. As a concrete example which makes use of this feature, we give an analytic description of the phenomenology of a constrained NMSSM close to a Peccei-Quinn symmetry point. The related pseudo-Goldstone boson appears in decays of the Higgs bosons and possibly of the lightest neutralino, and itself decays into (b anti-b) and (tau anti-tau).

  19. Dandy-Walker Malformation Presenting with Psychological Manifestations

    PubMed Central

    Dahanayake, Dulangi Maneksha Amerasinghe

    2016-01-01

    Dandy-Walker malformation, which is a congenital malformation of the cerebellum, is documented in literature to be associated with psychotic symptoms, obsessive compulsive symptoms, mood symptoms, hyperactivity, and impulsive behavior. The pathogenesis of psychiatric symptoms in Dandy-Walker malformation is thought to be due to disruption of the corticocerebellar tracts, resulting in what is known as cerebellar cognitive affective syndrome. We present a case of Dandy-Walker malformation presenting with psychiatric symptoms. This case highlights the necessity to be aware of psychiatric manifestations of cerebellar disease as it has an impact on the diagnosis and treatment. PMID:27493822

  20. Central nervous system manifestations of neonatal lupus: a systematic review.

    PubMed

    Chen, C C; Lin, K-L; Chen, C-L; Wong, A May-Kuen; Huang, J-L

    2013-12-01

    Neonatal lupus is a rare and acquired autoimmune disease. Central nervous system abnormalities are potential manifestations in neonatal lupus. Through a systematic literature review, we analyzed the clinical features of previously reported neonatal lupus cases where central nervous system abnormalities had been identified. Most reported neonatal lupus patients with central nervous system involvement were neuroimaging-determined and asymptomatic. Only seven neonatal lupus cases were identified as having a symptomatic central nervous system abnormality which caused physical disability or required neurosurgery. A high percentage of these neurosymptomatic neonatal lupus patients had experienced a transient cutaneous skin rash and had no maternal history of autoimmune disease before pregnancy.

  1. Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis

    PubMed Central

    Koksal, Deniz; Koksal, Aydin Seref; Gurakar, Ahmet

    2016-01-01

    Abstract Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic liver disease caused by diffuse inflammation, destruction and fibrosis of the intrahepatic bile ducts, ultimately leading to cirrhosis, portal hypertension and liver failure. The pathogenesis of PBC is incompletely understood, but current data suggest roles for genetic susceptibility and environmental factors. PBC is often thought of as an organ-specific autoimmune disease, which mainly targets the liver; however, lung tissue is also a site for autoimmune involvement of PBC. The pulmonary manifestations of PBC include abnormalities in gas transfer and pulmonary function, subclinical alveolitis, interstitial lung disease, granulomatous lung disease, airway disease, pulmonary hypertension, pulmonary hemorrhage and pleural effusion. PMID:27777894

  2. Atypical giant haemangioma of liver with systemic inflammatory manifestations.

    PubMed

    Khalid, Mohd; Ahmad, Mehtab; Jain, Amit; Rizvi, Imran

    2013-01-25

    Haemangioma is the most common benign tumour of the liver. Most of them are small in size (less than 4 cm) and are often asymptomatic and discovered incidentally on modern diagnostic imaging. Lesions with a diameter larger than 4 cm are called giant haemangiomas, and these are usually located in the right hepatic lobe. Although haemangioma is the most frequent benign tumour of the liver, 'giant' haemangiomas are rare. Such lesions may give rise to symptoms requiring treatment. In this case report, we describe the case of a 50-year-old Indian man who presented with giant liver haemangioma and systemic inflammatory manifestations.

  3. Pycnodysostosis: A bone dysplasia with unusual oral manifestation.

    PubMed

    Balaji, M S Anandi; Sundaram, R Kanmani Shanmuga; Karthik, Poornima; Asokan, Kannan

    2014-10-01

    Pycnodysostosis, a sclerosing bone dysplasia, is a rare autosomal recessive disorder with an estimated prevalence rate of one in one million. Patients with pycnodyostosis usually have normal intelligence, sexual development and life span. This condition is characterized by increased bone density and fragility along with oral manifestations like malposition teeth, hypoplastic maxilla, receded chin and delayed eruption of permanent teeth with discharging sinuses in the jaws because of poor blood supply. This is one such rare case report of a 47-year-old patient presenting with a complaint of fractured jaw and reviewing the clinical and radiographic characteristics of pycnodysostosis. PMID:25565757

  4. Extra intestinal manifestations and complications in inflammatory bowel disease.

    PubMed

    Marineaţă, Anca; Rezuş, Elena; Mihai, Cătălina; Prelipcean, Cristina Cijevschi

    2014-01-01

    Inflammatory bowel disease (IBD), including ulcerative colitis (UC) and Crohn's disease (CD), doesn't affect only the intestinal tract, but also involve other organs such as: eyes, skin, joints, liver and biliary tracts, kidneys, lungs, vascular system. It is difficult to differentiate the true extraintestinal manifestations from secondary extraintestinal complications. The pathogenetic autoimmune mechanisms include genetic susceptibility, antigenic display of autoantigen, aberrant self-recognition and immunopathogenetic autoantibodies against organ-specific cellular antigens shared by colon and extra-colonic organs. An important role is owned by microbes due to molecular mimicry. This paper reviews the frequency, clinical presentation and therapeutic implications of extraintestinal symptoms in inflammatory bowel diseases. PMID:25076688

  5. [The clinical and serological manifestations of Lyme disease in Russia].

    PubMed

    Anan'eva, L P; Skripnikova, I A; Barskova, V G; Steere, A C

    1995-01-01

    Out of 86 Lyme's disease patients with a history of migrating erythema nervous system, cardiovascular and articular involvement was observed in 27, 6 and 43% of cases. Acrodermatitis was diagnosed in 2% of patients. Affection of locomotor system manifested with acute arthritis episodes or pains in major joints. 11 patients of 12 examined at arthritis onset showed elevated titer of anti-Borrelia IgG antibodies. Serologically, of 80 patients with arthritis or arthralgia without prior migrating erythema 6 demonstrated antibodies to 5 and more Borrelia polypeptides.

  6. Beckwith-Wiedemann syndrome with overlapping Perlman syndrome manifestation.

    PubMed

    Ferianec, Vladimír; Bartova, Michaela

    2014-10-01

    Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome known as exomphalos-macroglossia - gigantism syndrome. Prognosis is good, prenatal diagnosis is important for pregnancy management but might be difficult due to clinical overlap with other syndromes. Perlman syndrome is an overgrowth syndrome with high perinatal mortality, most frequent antenatal findings include polyhydramnios, macrosomia, visceromegaly, nephromegaly and foetal ascites. Authors present a case of prenatally diagnosed BWS with severe ascites as first antenatal finding and lethal course, signs more typical of Perlman syndrome. This combination of clinical signs has not been published yet and may contribute to specification of possible prenatal manifestation of BWS. PMID:24215131

  7. Gastrointestinal, Pancreatic, and Hepatobiliary Manifestations of Cystic Fibrosis.

    PubMed

    Sathe, Meghana Nitin; Freeman, Alvin Jay

    2016-08-01

    Pulmonary disease is the primary cause of morbidity and mortality in people with cystic fibrosis (CF), but significant involvement within gastrointestinal, pancreatic, and hepatobiliary systems occurs as well. As in the airways, defects in CFTR alter epithelial surface fluid, mucus viscosity, and pH, increasing risk of stasis through the various hollow epithelial-lined structures of the gastrointestinal tract. This exerts secondary influences that are responsible for most gastrointestinal, pancreatic, and hepatobiliary manifestations of CF. Understanding these gastrointestinal morbidities of CF is essential in understanding and treating CF as a multisystem disease process and improving overall patient care. PMID:27469182

  8. [Intrathoracic lymphadenopathy: an unusual manifestation of rheumatoid arthritis].

    PubMed

    Khammassi, N; Bayouth, A; Abdelhedi, H; Balhouane, I; Hergli, I; Cherif, O

    2012-02-01

    Lung disease is the most frequent extra-articular manifestation of rheumatoid arthritis. It is detected in nearly 50% of patients with this multisystem affection, his knowledge has benefited from advances in computed tomography (CT). The inflammation can affect the pleura, the airways and the lung parenchyma. Intrathoracic lymphadenopathy complicating rheumatoid lung are not usual, and then pose the problem of differential diagnosis. We report a 51-year-old man, with a history of tobacco intoxication, suffering from rheumatoid arthritis who developed an interstitial lung disease at stage of fibrosis with mediastinal and hilar adenopathy. We will discuss the clinical, paraclinical, evolutionary and therapeutic particularities case.

  9. Oral mucosal manifestations in some genodermatoses: correlation with cutaneous lesions.

    PubMed

    Nico, Marcello Menta Simonsen; Hammerschmidt, Mariana; Lourenço, Silvia Vanessa

    2013-01-01

    The clinical picture of several genetic skin diseases may include the presence of oral mucosal lesions. These manifestations, however, have not been granted much attention in most dermatological publications. In this article, we fully review the oral mucosal lesions of tuberous sclerosis, dyskeratosis congenita, lipoidoproteinosis, Cowden disease, Darier's disease and pachyonychya congenita and compare these with their respective cutaneous lesions. Some dental aspects are discussed as well. This unifying approach may allow a better understanding of these oral lesions, avoiding obscure nomenclature and classification. PMID:24001555

  10. Automated Detection of Anomalous Shipping Manifests to Identify Illicit Trade

    SciTech Connect

    Sanfilippo, Antonio P.; Chikkagoudar, Satish

    2013-11-12

    We describe an approach to analyzing trade data which uses clustering to detect similarities across shipping manifest records, classification to evaluate clustering results and categorize new unseen shipping data records, and visual analytics to provide to support situation awareness in dynamic decision making to monitor and warn against the movement of radiological threat materials through search, analysis and forecasting capabilities. The evaluation of clustering results through classification and systematic inspection of the clusters show the clusters have strong semantic cohesion and offer novel ways to detect transactions related to nuclear smuggling.

  11. Chronic dermal sinuses as a manifestation of histiocytosis X.

    PubMed Central

    Sacks, S H; Hall, I; Ragge, N; Pritchard, J

    1986-01-01

    Two young patients presented with generalised lymphadenopathy, otorrhoea, otitis, and rash. Over the next few years chronically discharging sinuses began to form over enlarged nodes and histological appearances were typical of histiocytosis X. In neither case were micro-organisms isolated from the lesions, and in both patients healing occurred with immunosuppressive agents. Chronic dermal sinus formation secondary to lymph node disease has never before been recorded as a manifestation of histiocytosis X. Histiocytosis X should therefore be considered in the differential diagnosis of "suppurative" lymphadenopathy so that appropriate treatment may be given without delay. Images Case 1 PMID:3084014

  12. Brain abscess as a manifestation of spinal dermal sinus

    PubMed Central

    Emami-Naeini, Parisa; Mahdavi, Ali; Ahmadi, Hamed; Baradaran, Nima; Nejat, Farideh

    2008-01-01

    Dermal sinuses have been associated with a wide spectrum of clinical manifestations ranging from asymptomatic to drainage of purulent material from the sinus tract, inclusion tumors, meningitis, and spinal abscess. To date, there has been no documented report of brain abscess as a complication of spinal dermal sinus. Here, we report an 8-month-old girl who was presented initially with a brain abscess at early infancy but lumbar dermal sinus and associated spinal abscess were discovered afterwards. The probable mechanisms of this rare association have been discussed. PMID:19209295

  13. Esophageal Candidiasis as the Initial Manifestation of Acute Myeloid Leukemia.

    PubMed

    Komeno, Yukiko; Uryu, Hideki; Iwata, Yuko; Hatada, Yasumasa; Sakamoto, Jumpei; Iihara, Kuniko; Ryu, Tomiko

    2015-01-01

    A 47-year-old woman presented with persistent dysphagia. A gastroendoscopy revealed massive esophageal candidiasis, and oral miconazole was prescribed. Three weeks later, she returned to our hospital without symptomatic improvement. She was febrile, and blood tests showed leukocytosis (137,150 /μL, blast 85%), anemia and thrombocytopenia. She was diagnosed with acute myeloid leukemia (AML). She received chemotherapy and antimicrobial agents. During the recovery from the nadir, bilateral ocular candidiasis was detected, suggesting the presence of preceding candidemia. Thus, esophageal candidiasis can be an initial manifestation of AML. Thorough examination to detect systemic candidiasis is strongly recommended when neutropenic patients exhibit local candidiasis prior to chemotherapy.

  14. Chronic Eosinophilic Leukemia Presenting Predominantly with Cutaneous Manifestations.

    PubMed

    Vidyadharan, Suja; Joseph, Bebisha; Nair, Sukumaran Pradeep

    2016-01-01

    A 37-year-old male presented with severe oral and genital mucosal ulcers, lichenoid eruption and twenty-nail dystrophy. Systemic examination was normal, except for anemia. On investigations, he was found to have persistently elevated peripheral eosinophilia, absolute eosinophil count >5000/mm(3), bone marrow showing increased eosinophilic precursors, and infiltration by atypical cells. The serum vitamin B12 levels were grossly elevated, and Philadelphia chromosome study was negative. Thus, a diagnosis of chronic eosinophilic leukemia was made. The patient showed excellent response to imatinib mesylate. We are reporting a rare type of leukemia presenting with predominantly cutaneous manifestations. PMID:27512192

  15. The oral manifestations of intestinal lymphangiectasia: case report.

    PubMed

    Ralph, P M; Troutman, K C

    1996-01-01

    Intestinal lymphangiectasia is a rare autosomal dominant disorder or acquired condition that leads to lymph obstruction, poor chyle transport, and concomitant problems of hypoproteinemia, lymphocytopenia, hypogammaglobulinemia, and peripheral edema. Patients develop diarrhea, steatorrhea, and hypocalcemia secondary to fat-soluble vitamin malabsorption. Treatment is a restrictive diet of low fat, medium chain triglycerides. Oral manifestations are gingivitis due to poor PMN function and enamel defects due to poor calcium absorption. A case of a 14-year-old boy with both gingival and enamel problems secondary to intestinal lymphangiectasia is reported. PMID:8970209

  16. Cholangitis as an initial manifestation of polyarteritis nodosa.

    PubMed

    Kanai, Risa; Nakamura, Masamoto; Tomisato, Kota; Fukuhara, Tomofumi; Kondo, Akiyuki; Nakamura, Shoko; Matsukawa, Shinobu; Yabutani, Akira; Kobashikawa, Kasen; Nakayoshi, Tomokuni; Uchima, Nobufumi; Kosuge, Noritake; Yoshimi, Naoki

    2014-01-01

    A previously healthy 89-year-old man was admitted to our hospital with right upper quadrant pain and mild fever. A diagnosis of cholangitis was suspected based on the patient's physical findings and imaging features. Although he received treatment typical for cholangitis, he suddenly died of shock for unknown reasons two months after disease onset. An autopsy revealed a ruptured hepatic artery aneurysm, which had caused lethal intra-abdominal bleeding. In addition, systemic necrotizing vasculitis of small- and medium-sized arteries was detected, and polyarteritis nodosa (PAN) was diagnosed after the autopsy. Biliary symptoms as the initial manifestation of PAN are extremely rare.

  17. Ocular manifestations in a child with systemic brucellosis

    PubMed Central

    Mohammadi, Zahra; Dehghani, Alireza; Ghanbari, Heshmat Ollah; Akhlaghi, Mohammad Reza; Nasrollahi, Kobra; Salam, Hasan

    2014-01-01

    Brucellosis is a zoonotic disease with widespread prevalence. It presents with in various range and often with the presence of non-specific clinical signs and symptoms. Brucellosis also may cause different manifestations in eyes such as uveitis, keratitis, conjunctivitis and neuro-ophthalmic defects. Ocular brucellosis is rare among children. Herein, we present a 7-year-old girl with systemic and ocular brucellosis. After treatment with systemic steroid and antibiotics, her signs and symptoms disappeared. Since early treatment is important in preventing permanent visual loss and the other complications of ocular brucellosis, examination of the eyes in brucellosis patients must always be noticed by clinicians working in this field. PMID:25364370

  18. Haemoptysis as a primary manifestation of cryptogenic organizing pneumonia (COP).

    PubMed

    Chatzivasiloglou, Fotini; Katsenos, Stamatis; Psathakis, Konstantinos; Tsintiris, Konstantinos

    2015-01-01

    Cryptogenic organizing pneumonia (COP), previously called bronchiolitis obliterans organizing pneumonia (BOOP) is a clinicopathological disorder of unknown aetiology but increasingly reported. It usually presents with symptoms of dyspnea, cough, fever, weight loss accompanied by the presence of alveolar opacities on chest radiograph. Haemoptysis, described as blood streaking has only rarely been reported as primary presentation of COP. Herein, we report a case of COP in which submassive haemoptysis was the main clinical manifestation. The clinical, radiological, pathological, and therapeutic aspects of the disease are briefly discussed. Cryptogenic organizing pneumonia should be taken into consideration in the differential diagnosis of severe haemoptysis. PMID:27451594

  19. Extraparenchymal (Racemose) Neurocysticercosis and Its Multitude Manifestations: A Comprehensive Review

    PubMed Central

    Mehta, Anish; Rangasetty, Srinivasa

    2015-01-01

    Neurocysticercosis is an infection of the central nervous system caused by the larval form of the pork tapeworm Taenia solium. In the brain it occurs in two forms: parenchymal and extraparenchymal or racemose cysts. The clinical presentation of racemose cysts is pleomorphic, and is quite different from parenchymal cysticercosis. The clinical diagnosis of racemose cysts is quite challenging, with neuroimaging being the mainstay. However, the advent of newer brain imaging modalities has made a more accurate diagnosis possible. The primary focus of this article is racemose neurocysticercosis and its multitude manifestations, and includes a discussion of the newer diagnostic modalities and treatment options. PMID:26022457

  20. Central nervous system manifestations of Angiostrongylus cantonensis infection

    PubMed Central

    Martins, Yuri C.; Tanowitz, Herbert B.; Kazacos, Kevin R.

    2014-01-01

    Over 20 species of Angiostrongylus have been described from around the world, but only Angiostrongylus cantonensis has been confirmed to cause central nervous system disease in humans. A neurotropic parasite that matures in the pulmonary arteries of rats, A. cantonensis is the most common cause of eosinophilic meningitis in southern Asia and the Pacific and Caribbean islands. The parasite can also cause encephalitis/encephalomyelitis and rarely ocular angiostrongyliasis. The present paper reviews the life cycle, epidemiology, pathogenesis, clinical features, diagnosis, treatment, prevention and prognosis of A. cantonesis infection. Emphasis is given on the spectrum of central nervous system manifestations and disease pathogenesis. PMID:25312338

  1. Spectroscopic manifestation of stretching vibrations of glycosidic linkage in polysaccharides

    NASA Astrophysics Data System (ADS)

    Nikonenko, N. A.; Buslov, D. K.; Sushko, N. I.; Zhbankov, R. G.

    2005-10-01

    Manifestation of stretching vibrations of glycosidic linkage in the infrared spectra of polysaccharides (native, microcrystalline, mercerized celluloses, amylose, starches) has been studied using the regularized method of deconvolution. It has been shown that the glycosidic linkage formation in the polysaccharides is characterized by the appearance of new absorption bands in the 1175-1140 cm -1 range as compared to their corresponding monomers. In the 1000-920 cm -1 region differences between the infrared spectra of polysaccharides due to the changes in the glycosidic linkage configuration have been found.

  2. Spectrum of X-ray manifestations in Cockayne's syndrome

    SciTech Connect

    Bensman, A.; Faure, C.; Kaufmann, H.J.

    1981-12-01

    Cockayne's syndrome is a rare familial disorder characterised clinically by premature aging, appearing progressively from the third year on. The radiological manifestations of eight affected children have been studied and summarised. It is concluded that a skeletal survey can provide a roentgenologic pattern suggesting the diagnosis, even when it is inconclusive from the clinical signs during the first years of life. The aetiology of this syndrome is unknown, but the authors postulate the possible role of a defect of thymic hormone which has been found in all their cases.

  3. Extraparenchymal (Racemose) Neurocysticercosis and Its Multitude Manifestations: A Comprehensive Review.

    PubMed

    Mahale, Rohan R; Mehta, Anish; Rangasetty, Srinivasa

    2015-07-01

    Neurocysticercosis is an infection of the central nervous system caused by the larval form of the pork tapeworm Taenia solium. In the brain it occurs in two forms: parenchymal and extraparenchymal or racemose cysts. The clinical presentation of racemose cysts is pleomorphic, and is quite different from parenchymal cysticercosis. The clinical diagnosis of racemose cysts is quite challenging, with neuroimaging being the mainstay. However, the advent of newer brain imaging modalities has made a more accurate diagnosis possible. The primary focus of this article is racemose neurocysticercosis and its multitude manifestations, and includes a discussion of the newer diagnostic modalities and treatment options. PMID:26022457

  4. Osteoskeletal manifestations of scurvy: MRI and ultrasound findings.

    PubMed

    Polat, Ahmet Veysel; Bekci, Tumay; Say, Ferhat; Bolukbas, Emrah; Selcuk, Mustafa Bekir

    2015-08-01

    Scurvy has become very rare in the modern world. The incidence of scurvy in the pediatric population is extremely low. In the pediatric population, musculoskeletal manifestations are more common and multiple subperiosteal hematomas are an important indicator for the diagnosis of scurvy. Although magnetic resonance imaging findings of scurvy are well described in the literature, to our knowledge, ultrasound findings have not yet been described. In this article, we report a case of scurvy with associated magnetic resonance imaging and ultrasound findings. PMID:25597047

  5. Manifestation of resonance-related chaos in coupled Josephson junctions

    NASA Astrophysics Data System (ADS)

    Shukrinov, Yu. M.; Hamdipour, M.; Kolahchi, M. R.; Botha, A. E.; Suzuki, M.

    2012-11-01

    Manifestation of chaos in the temporal dependence of the electric charge is demonstrated through the calculation of the maximal Lyapunov exponent, phase-charge and charge-charge Lissajous diagrams and correlation functions. It is found that the number of junctions in the stack strongly influences the fine structure in the current-voltage characteristics and a strong proximity effect results from the nonperiodic boundary conditions. The observed resonance-related chaos exhibits intermittency. The criteria for a breakpoint region with no chaos are obtained. Such criteria could clarify recent experimental observations of variations in the power output from intrinsic Josephson junctions in high temperature superconductors.

  6. Crohn's disease manifesting in the head and neck.

    PubMed

    Bradley, Patrick J; Ferlito, Alfio; Devaney, Kenneth O; Rinaldo, Alessandra

    2004-04-01

    Crohn's disease may present to the head and neck surgeon with symptoms and signs attributed to the disease. Many clinical presentations, both specific and non-specific, have been recorded in the oral cavity, nose and larynx. Some of these clinical manifestations have been found to be consistent with Crohn's disease, but most have been attributed to Crohn's disease without histologic confirmation of the presence of non-caseating granulomas, which is pathognomonic of Crohn's disease. Lesions, when present without an associated diagnosis of Crohn's disease, should be ascribed the diagnosis of orofacial granulomatosis, until a diagnosis of intestinal Crohn's disease has been confirmed.

  7. Pycnodysostosis: A bone dysplasia with unusual oral manifestation

    PubMed Central

    Balaji, M S Anandi; Sundaram, R Kanmani Shanmuga; Karthik, Poornima; Asokan, Kannan

    2014-01-01

    Pycnodysostosis, a sclerosing bone dysplasia, is a rare autosomal recessive disorder with an estimated prevalence rate of one in one million. Patients with pycnodyostosis usually have normal intelligence, sexual development and life span. This condition is characterized by increased bone density and fragility along with oral manifestations like malposition teeth, hypoplastic maxilla, receded chin and delayed eruption of permanent teeth with discharging sinuses in the jaws because of poor blood supply. This is one such rare case report of a 47-year-old patient presenting with a complaint of fractured jaw and reviewing the clinical and radiographic characteristics of pycnodysostosis. PMID:25565757

  8. Tracheobronchitis as an extraintestinal manifestation of ulcerative colitis

    PubMed Central

    Javia, Siddharth; Agrawal, Abhinav; Patell, Rushad; Jasdanwala, Sarfaraz

    2014-01-01

    Respiratory involvement is a rare extraintestinal manifestation of ulcerative colitis (UC). It commonly presents as bronchiectasis and rarely as tracheobronchitis. It can occur before or after the presentation of gastrointestinal symptoms. Only rarely does it occur after the patient undergoes colectomy. Diagnosis should be considered in any patient with UC who presents with unexplained upper respiratory symptoms and a negative work up for infectious aetiologies. It responds well to immunosuppressive therapy. We present a case of a 21-year-old woman who underwent colectomy for ulcerative colitis and later presented with new onset severe reversible inflammation of the upper respiratory tract. PMID:25326560

  9. [Congenital deficiency of protein C with late clinical manifestation].

    PubMed

    Ortega Calvo, M; Méndez Mora, J L; Macías Pérez, V; Mellado Fuentes, F

    1994-07-01

    The congenital deficit of protein C has a great diversity of clinical manifestations regarding age. In this paper, we describe the case of a man whose initial symptomatology appeared at the age of 68. Protein C is a vitamin-K dependent plasmatic glycoprotein which has anticoagulant activity through the inactivation of factors Va and VIIIa. The hereditary deficit of protein C is usually presented in a dominant autosomic mode with partial penetration and its prevalence is estimated in each of 200-300 healthy blood donors. PMID:7981364

  10. Chronic Eosinophilic Leukemia Presenting Predominantly with Cutaneous Manifestations

    PubMed Central

    Vidyadharan, Suja; Joseph, Bebisha; Nair, Sukumaran Pradeep

    2016-01-01

    A 37-year-old male presented with severe oral and genital mucosal ulcers, lichenoid eruption and twenty-nail dystrophy. Systemic examination was normal, except for anemia. On investigations, he was found to have persistently elevated peripheral eosinophilia, absolute eosinophil count >5000/mm3, bone marrow showing increased eosinophilic precursors, and infiltration by atypical cells. The serum vitamin B12 levels were grossly elevated, and Philadelphia chromosome study was negative. Thus, a diagnosis of chronic eosinophilic leukemia was made. The patient showed excellent response to imatinib mesylate. We are reporting a rare type of leukemia presenting with predominantly cutaneous manifestations. PMID:27512192

  11. Tuberous sclerosis with oral manifestations: A rare case report

    PubMed Central

    Sodhi, SPS; Dang, Ramandeep Singh; Brar, Gursimrat

    2016-01-01

    Tuberous sclerosis complex (TSC) is a neurocutaneous syndrome, inherited as an autosomal dominant trait with a high incidence of sporadic cases and protean clinical expression, with a incidence of prevalence between 1 in 10,000 and 1 in 170,000. The cardinal features of TSC are skin lesions, convulsive seizures, and mental retardation. We report a sporadically occurring case of definite TSC in a young female who presented with oral and cutaneous manifestations without mental retardation or history of convulsive seizures, which to the best of our knowledge has not been reported so far. PMID:26958526

  12. Chromosomal and Multifactorial Genetic Disorders with Oral Manifestations

    PubMed Central

    Patil, Shankargouda; Rao, Roopa S; Majumdar, Barnali

    2014-01-01

    The chromosomal disorders are individually rare, but collectively they are common whereas the multifactorial disorders are the most common form of genetic disorders. The chromosomal anomalies typically arise from alterations in the DNA containing chromosomal regions and can be reliably detected by karyotype analysis, whereas the multifactorial disorders demonstrate multi-gene as well as environmental interactions. Both the chromosomal and multifactorial disorders may manifest signs and symptoms such as a combination of birth defects, physical disabilities, challenging behavior and certain craniofacial defects as well, the knowledge of which can aid in a better patient management in everyday practice of dentistry. PMID:25395808

  13. Imaging Manifestations of Hematologic Diseases with Renal and Perinephric Involvement.

    PubMed

    Purysko, Andrei S; Westphalen, Antonio C; Remer, Erick M; Coppa, Christopher P; Leão Filho, Hilton M; Herts, Brian R

    2016-01-01

    The kidneys and perinephric tissues can be affected by a variety of hematologic disorders, which usually occur in the setting of multisystem involvement. In many of these disorders, imaging is used to evaluate the extent of disease, guide biopsy, and/or monitor disease activity and patient response to therapy. Lymphoma, leukemia, and multiple myeloma commonly manifest as multiple parenchymal or perinephric lesions. Erdheim-Chester disease and Rosai-Dorfman disease, rare forms of multisystemic histiocytosis, are often identified as perinephric and periureteral masses. Renal abnormalities depicted at imaging in patients with sickle cell disease include renal enlargement, papillary necrosis, and renal medullary carcinoma. Sickle cell disease, along with other causes of intravascular hemolysis, can also lead to hemosiderosis of the renal cortex. Thrombosis of renal veins is sometimes seen in patients with coagulation disorders but more often occurs in association with certain malignancies and nephrotic syndrome. Immunoglobulin G4-related sclerosing disease is another multisystem process that often produces focal renal lesions, seen along with involvement of more characteristic organs such as the pancreas. Perinephric lesions with calcifications should raise the possibility of secondary amyloidosis, especially in patients with a history of lymphoma and multiple myeloma. Although the imaging patterns of renal and perinephric involvement are usually not specific for a single entity, and the same entity can manifest with different or overlapping patterns, familiarity with these patterns and key clinical and histopathologic features may help to narrow the differential diagnosis and determine the next step of care. (©)RSNA, 2016. PMID:27257766

  14. [Nephrotic syndrome as the first manifestation of juvenile systemic scleroderma.

    PubMed

    Couto, Saulo B; Sallum, Adriana M; Henriques, Luciana S; Malheiros, Denise M; Silva, Clovis A; Vaisbich, Maria H

    2014-10-22

    Renal involvement occurs in 1%-12% in juvenile systemic sclerosis (JSSc) patients, mainly with arterial hypertension, proteinuria and scleroderma renal crisis. We report herein a patient who presented nephrotic syndrome (NS) as the first manifestation of JSSc with focal segmental glomerulosclerosis (FSGS). A female patient presented steroid-sensitive NS at the age of 12 years. At 14 years, she had orbital and lower limbs edema, arterial hypertension, sclerodactyly and proximal skin sclerosis. Moderate capillary dilation and mild focal devascularization were observed in nailfold capillaroscopy, compatible with early stage of scleroderma (scleroderma pattern). Percutaneous renal biopsy guided by ultrasound revealed focal segmental glomerulosclerosis and direct immunofluorescence were negative. Therefore, she fullfilled the provisional classification criteria for JSSc. Patient was treated with oral 25-hydroxyvitamin D (800 IU/day), methotrexate (0.5mg/kg/week) and amlodipin (0.15 mg/kg). Prednisone (60 mg/m(2)/day) was administered for 4 consecutive weeks, followed by alternate-day (40mg/m(2)) for 2 consecutive months, with tapering for 4 months and then stopping this medication. Currently she is being treated with methotrexate 15 mg/week, without edema and proteinuria. In conclusion, we reported a rare case of NS with FSGS as the first manifestation of scleroderma. Therefore, renal biopsy is mandatory in JSSc patients with sustained proteinuria or NS.

  15. [Skin manifestations of toxic syndrome due to denatured rapeseed oil].

    PubMed

    Fonseca, E

    2009-12-01

    This article offered an extensive description of the clinical and pathological features and time-course of the skin manifestations of toxic syndrome caused by denatured rapeseed oil, also known as toxic oil syndrome. This new condition occurred in Spain in 1981 and was due to the ingestion of rapeseed oil intended for industrial use that had been denatured with anilines and subsequently refined and sold fraudulently as olive oil. In total, 20,000 cases and 400 deaths were reported. The disease affected mainly women, particularly in the late stages. In the acute phase, the predominant skin manifestations were toxic-allergic rashes reminiscent of allergic urticaria in the dermatopathologic study. In approximately 25% of cases, the patients' skin subsequently took on an edematous appearance, with pigmentary abnormalities shown to be related to cutaneous mucinosis. Finally, a characteristic sclerodermatous condition would develop that tended to improve spontaneously. The constant presence of mast cells in all biopsies and the development of mastocytosis in several patients pointed to an important role for these cells in the pathogenesis of the condition. This was subsequently confirmed in other sclerodermatous processes. In 1989, eosinophilia-myalgia syndrome caused by toxins present in tryptophan food supplements was reported in the United States. This syndrome resembled toxic oil syndrome in many ways and demonstrated that mucinosis and toxic sclerodermatous processes do exist.

  16. T Helper Cell Subsets in Clinical Manifestations of Psoriasis

    PubMed Central

    Diani, Marco; Altomare, Gianfranco

    2016-01-01

    Psoriasis is a chronic inflammatory skin disease, which is associated with systemic inflammation and comorbidities, such as psoriatic arthritis and cardiovascular diseases. The autoimmune nature of psoriasis has been established only recently, conferring a central role to epidermal CD8 T cells recognizing self-epitopes in the initial phase of the disease. Different subsets of helper cells have also been reported as key players in the psoriasis pathogenesis. Here, we reviewed the knowledge on the role of each subset in the psoriatic cascade and in the different clinical manifestations of the disease. We will discuss the role of Th1 and Th17 cells in the initiation and in the amplification phase of cutaneous inflammation. Moreover, we will discuss the recently proposed role of tissue resident Th22 cells in disease memory in sites of recurrent psoriasis and the possible involvement of Th9 cells. Finally, we will discuss the hypothesis of a link between T helper cell subsets recirculating from the skin and the systemic manifestations of psoriasis. PMID:27595115

  17. Initial clinical manifestations of Parkinson's disease: features and pathophysiological mechanisms.

    PubMed

    Rodriguez-Oroz, Maria C; Jahanshahi, Marjan; Krack, Paul; Litvan, Irene; Macias, Raúl; Bezard, Erwan; Obeso, José A

    2009-12-01

    A dopaminergic deficiency in patients with Parkinson's disease (PD) causes abnormalities of movement, behaviour, learning, and emotions. The main motor features (ie, tremor, rigidity, and akinesia) are associated with a deficiency of dopamine in the posterior putamen and the motor circuit. Hypokinesia and bradykinesia might have a dual anatomo-functional basis: hypokinesia mediated by brainstem mechanisms and bradykinesia by cortical mechanisms. The classic pathophysiological model for PD (ie, hyperactivity in the globus pallidus pars interna and substantia nigra pars reticulata) does not explain rigidity and tremor, which might be caused by changes in primary motor cortex activity. Executive functions (ie, planning and problem solving) are also impaired in early PD, but are usually not clinically noticed. These impairments are associated with dopamine deficiency in the caudate nucleus and with dysfunction of the associative and other non-motor circuits. Apathy, anxiety, and depression are the main psychiatric manifestations in untreated PD, which might be caused by ventral striatum dopaminergic deficit and depletion of serotonin and norepinephrine. In this Review we discuss the motor, cognitive, and psychiatric manifestations associated with the dopaminergic deficiency in the early phase of the parkinsonian state and the different circuits implicated, and we propose distinct mechanisms to explain the wide clinical range of PD symptoms at the time of diagnosis. PMID:19909911

  18. Nasopharyngeal Tuberculosis: Epidemiology, Mechanism of Infection, Clinical Manifestations, and Management

    PubMed Central

    Sittitrai, Pichit

    2016-01-01

    Nasopharyngeal tuberculosis (NPTB) is a noteworthy disease especially in its worldwide spread of the Mycobacterium infection. Although NPTB has been identified in less than one percent of TB cases, recent multiple case reports indicate an either increased awareness or incidence of this disease. The most helpful diagnostic tool is an uncomplicated nasopharyngeal biopsy. However, NPTB is usually ignored because it has varied clinical manifestations and similar presentations with other more common head and neck diseases. Furthermore, the most common presenting symptom is cervical lymphadenopathy mimicking nasopharyngeal carcinoma, a more common and serious disease. Treatment outcomes of NPTB are good in both HIV-positive or HIV-negative patients. In addition, pulmonary tuberculosis association was reported in wide range between 8.3% and 82% which should be considered in a treatment program. In conclusion, early diagnosis and management in NPTB can be achieved by (1) increased awareness of this disease, (2) improvement in knowledge regarding clinical manifestations, and (3) improvement of diagnostic techniques. PMID:27034677

  19. T Helper Cell Subsets in Clinical Manifestations of Psoriasis

    PubMed Central

    Diani, Marco; Altomare, Gianfranco

    2016-01-01

    Psoriasis is a chronic inflammatory skin disease, which is associated with systemic inflammation and comorbidities, such as psoriatic arthritis and cardiovascular diseases. The autoimmune nature of psoriasis has been established only recently, conferring a central role to epidermal CD8 T cells recognizing self-epitopes in the initial phase of the disease. Different subsets of helper cells have also been reported as key players in the psoriasis pathogenesis. Here, we reviewed the knowledge on the role of each subset in the psoriatic cascade and in the different clinical manifestations of the disease. We will discuss the role of Th1 and Th17 cells in the initiation and in the amplification phase of cutaneous inflammation. Moreover, we will discuss the recently proposed role of tissue resident Th22 cells in disease memory in sites of recurrent psoriasis and the possible involvement of Th9 cells. Finally, we will discuss the hypothesis of a link between T helper cell subsets recirculating from the skin and the systemic manifestations of psoriasis.

  20. Extrahepatic manifestations of chronic hepatitis C virus infection

    PubMed Central

    Comarmond, Cloe; Domont, Fanny; Savey, Léa; Desbois, Anne C.; Saadoun, David

    2016-01-01

    During hepatitis C virus (HCV) chronic infection, extrahepatic manifestations are frequent and polymorphous. This article reports on a large cohort of patients with HCV-related autoimmune or lymphoproliferative disorders, from mixed cryoglobulinemia vasculitis to frank lymphomas. The relationship between HCV infection and such immune-related diseases has been formally demonstrated by epidemiological, clinical, immunological and pathological data, and results of therapeutic trials. More recently, other nonliver-related HCV disorders have been reported, including cardiovascular (i.e. stroke, ischemic heart disease), renal, metabolic and central nervous system diseases. For these manifestations, most evidence comes from large epidemiological studies; there is a need for mechanistic studies and therapeutic trials for confirmation. Beyond the risk of developing liver complications, that is, cirrhosis and liver cancer, patients with HCV infection have an increased risk of morbidity and mortality related to nonliver diseases. HCV chronic infection should be analyzed as a systemic disease in which extrahepatic consequences increase the weight of its pathological burden. The need for effective viral eradication measures is underlined. PMID:26862398

  1. Manifestations of paediatric Leishmania infantum infections in Malta.

    PubMed

    Pace, David; Williams, Thomas N; Grochowska, Alicja; Betts, Alexandra; Attard-Montalto, Simon; Boffa, Michael J; Vella, Cecil

    2011-01-01

    Leishmania infantum is endemic in the Maltese archipelago, a group of islands in the Mediterranean which are visited frequently by tourists from Northern European countries. The burden of leishmaniasis is highest in children who may present with cutaneous or visceral manifestations. We describe systematically the manifestations, diagnosis and management of leishmaniasis in children <14 years of age, who had a histopathological diagnosis of leishmaniasis in Malta, from 2004 to 2008. Eleven children were diagnosed with leishmaniasis; 8 children (15-44 months of age) had visceral disease and three (aged 9-13 years) suffered cutaneous infections. Prolonged high grade fever, pallor, hepatosplenomegaly, and pancytopenia were common presenting features of visceralisation. Diagnosis was based on the visualisation of amastigotes from bone marrow aspirates. Pentavalent antimonials were associated with treatment failure in two children, whilst liposomal amphotericin B was curative in all. Children with cutaneous leishmaniasis had dry crusted ulcero-nodular lesions on exposed areas which responded to intra-lesional instillation of sodium stibogluconate or to cryotherapy. Leishmaniasis should be included in the differential diagnosis of fever and hepatosplenomegaly or chronic cutaneous lesions in children who travel to Malta.

  2. What we know about ocular manifestations of Ebola.

    PubMed

    Moshirfar, Majid; Fenzl, Carlton R; Li, Zhan

    2014-01-01

    Ebola hemorrhagic fever is a deadly disease caused by several species of ebolavirus. The current outbreak of 2014 is unique in that it has affected a greater number of people than ever before. It also has an unusual geographic distribution. Nonspecific findings such as fever and generalized weakness have traditionally been very common early in the acute phase. Ophthalmic manifestations have also been reported in significant numbers. Conjunctival injection has been identified in both the acute and late phases. Subconjunctival hemorrhage and excessive lacrimation have also been reported. Various forms of uveitis have been associated with the convalescent phase of the disease. When identified in conjunction with other signs such as fever, acute findings such as conjunctivitis may contribute to the diagnosis of Ebola hemorrhagic fever. Ideally, serologic testing should be performed prior to isolation and treatment of these individuals. Considering the prevalence of the current outbreak and the threat of transcontinental spread, ophthalmic health professionals need to be aware of the ocular manifestations of Ebola hemorrhagic fever as well as the associated signs and symptoms in order to prevent further spread.

  3. Analysis of Recent Manifests for Goods Imported through US Ports

    SciTech Connect

    Descalle, M; Manatt, D; Slaughter, D

    2006-09-27

    Several active interrogation techniques are being developed to detect shielded special nuclear materials (SNM) hidden in cargo containers loaded on container ships arriving at US ports. It raises the questions of the types of cargos in which SNM could be hidden, and their impact on detected signatures. Since the definition of a set of ''typical'' or standard cargos has proven to be difficult, we analyzed shipping manifests for US imports shipped through North American ports collected on 14 days distributed over 12 months. From these data, we generated several distribution functions such as commodity categories, average densities, and packaging types that could be of interest to the cargo scanning community. One of the cargo scanning techniques currently under development at LLNL is based on neutron active interrogation, and relies on the unique signature of beta-delayed gammas emitted by fission products in the 3 to 7 MeV energy range. {sup 19}F(n,a){sup 16}N, has been identified as the main potential interference for 7 and 9 MeV neutron beams. Estimates of cargo compositions based on manifests identified as containing fluorine are presented.

  4. Initial cutaneous manifestations of Hutchinson-Gilford progeria syndrome.

    PubMed

    Rork, Jillian F; Huang, Jennifer T; Gordon, Leslie B; Kleinman, Monica; Kieran, Mark W; Liang, Marilyn G

    2014-01-01

    Hutchinson-Gilford progeria syndrome (HGPS) is a rare, uniformly fatal, premature aging disease with distinct dermatologic features. We sought to identify and describe the initial skin and hair findings as potential diagnostic signs of the disease. We performed a chart review of the structured initial intake histories of 39 individuals with HGPS enrolled in clinical trials from 2007 to 2010 at Boston Children's Hospital, limited to cutaneous history from birth to 24 months. Medical photographs were provided through the clinical trials and the Progeria Research Foundation Medical and Research Database at Brown University Center for Gerontology and Healthcare Research. All 39 patients reported skin and hair abnormalities within the first 24 months of life. Pathologies included sclerodermoid change, prominent superficial veins, dyspigmentation, and alopecia. The mean age of presentation for each finding was <12 months. The most frequently reported skin feature was sclerodermoid change, which commonly involved the abdomen and bilateral lower extremities. Prominent superficial vasculature manifested as circumoral cyanosis and pronounced veins on the scalp and body. Hypo- and hyperpigmentation were observed over areas of sclerodermoid change. Scalp alopecia progressed in a distinct pattern, with preservation of the hair over the midscalp and vertex areas for the longest period of time. HGPS has distinct cutaneous manifestations during the first 2 years of life that may be the first signs of disease. Awareness of these findings could expedite diagnosis.

  5. Dermatological Manifestations of Postural Tachycardia Syndrome Are Common and Diverse

    PubMed Central

    Deb, Anindita; Culbertson, Collin; Morgenshtern, Karen; DePold Hohler, Anna

    2016-01-01

    Background and Purpose Postural tachycardia syndrome (POTS) is a syndrome of orthostatic intolerance in the setting of excessive tachycardia with orthostatic challenge, and these symptoms are relieved when recumbent. Apart from symptoms of orthostatic intolerance, there are many other comorbid conditions such as chronic headache, fibromyalgia, gastrointestinal disorders, and sleep disturbances. Dermatological manifestations of POTS are also common and range widely from livedo reticularis to Raynaud's phenomenon. Methods Questionnaires were distributed to 26 patients with POTS who presented to the neurology clinic. They were asked to report on various characteristics of dermatological symptoms, with their answers recorded on a Likert rating scale. Symptoms were considered positive if patients answered with "strongly agree" or "agree", and negative if they answered with "neutral", "strongly disagree", or "disagree". Results The most commonly reported symptom was rash (77%). Raynaud's phenomenon was reported by over half of the patients, and about a quarter of patients reported livedo reticularis. The rash was most commonly found on the arms, legs, and trunk. Some patients reported that the rash could spread, and was likely to be pruritic or painful. Very few reported worsening of symptoms on standing. Conclusions The results suggest that dermatological manifestations in POTS vary but are highly prevalent, and are therefore of important diagnostic and therapeutic significance for physicians and patients alike to gain a better understanding thereof. Further research exploring the underlying pathophysiology, incidence, and treatment strategies is necessary. PMID:26610893

  6. Social Comparison Manifests in Event-related Potentials

    PubMed Central

    Luo, Yi; Feng, Chunliang; Wu, Tingting; Broster, Lucas S.; Cai, Huajian; Gu, Ruolei; Luo, Yue-jia

    2015-01-01

    Social comparison, a widespread phenomenon in human society, has been found to affect outcome evaluation. The need to belong to a social group may result in distinct neural responses to diverse social comparison outcomes. To extend previous studies by examining how social comparison with hierarchical characteristics is temporally processed, electroencephalography responses were recorded in the current study. Participants played a lottery game with two pseudo-players simultaneously and received both their own and the other two players’ outcomes. Results of three event-related potential components, including the P2, the feedback-related negativity (FRN), and the late positive component (LPC), indicate that social comparison manifests in three stages. First, outcomes indicating a different performance from others elicited a larger P2 than evenness. Second, the FRN showed hierarchical sensitivity to social comparison outcomes. This effect manifested asymmetrically. Finally, large difference between the participant’s outcome and the other two players’ evoked a larger LPC than the medium difference and the even condition. We suggest that during social comparison, people detect if there is any difference between self and others, and then evaluate the information of this difference hierarchically, and finally interpret the situations in which oneself deviates from the group as most motivationally salient. PMID:26183734

  7. Nasopharyngeal Tuberculosis: Epidemiology, Mechanism of Infection, Clinical Manifestations, and Management.

    PubMed

    Srivanitchapoom, Chonticha; Sittitrai, Pichit

    2016-01-01

    Nasopharyngeal tuberculosis (NPTB) is a noteworthy disease especially in its worldwide spread of the Mycobacterium infection. Although NPTB has been identified in less than one percent of TB cases, recent multiple case reports indicate an either increased awareness or incidence of this disease. The most helpful diagnostic tool is an uncomplicated nasopharyngeal biopsy. However, NPTB is usually ignored because it has varied clinical manifestations and similar presentations with other more common head and neck diseases. Furthermore, the most common presenting symptom is cervical lymphadenopathy mimicking nasopharyngeal carcinoma, a more common and serious disease. Treatment outcomes of NPTB are good in both HIV-positive or HIV-negative patients. In addition, pulmonary tuberculosis association was reported in wide range between 8.3% and 82% which should be considered in a treatment program. In conclusion, early diagnosis and management in NPTB can be achieved by (1) increased awareness of this disease, (2) improvement in knowledge regarding clinical manifestations, and (3) improvement of diagnostic techniques. PMID:27034677

  8. Clinical manifestations and distribution of cutaneous leishmaniasis in pakistan.

    PubMed

    Afghan, Abaseen Khan; Kassi, Masoom; Kasi, Pashtoon Murtaza; Ayub, Adil; Kakar, Niamatullah; Marri, Shah Muhammad

    2011-01-01

    Cutaneous leishmaniasis (CL) is a rising epidemic in Pakistan. It is a major public health problem in the country especially alongside regions bordering the neighboring Afghanistan and cities that have had the maximum influx of refugees. The purpose of our paper is to highlight the diverse clinical manifestations of the disease seen along with the geographic areas affected, where the hosts are particularly susceptible. This would also be helpful in presenting the broad spectrum of the disease for training of health care workers and help in surveillance of CL in the region. The increased clinical diversity and the spectrum of phenotypic manifestations noted underscore the fact that the diagnosis of CL should be not only considered when dealing with common skin lesions, but also highly suspected by dermatologists and even primary care physicians even when encountering uncommon pathologies. Hence, we would strongly advocate that since most of these patients present to local health care centers and hospitals, primary care practitioners and even lady health workers (LHWs) should be trained in identification of at least the common presentations of CL. PMID:22174721

  9. Case report: bipolar disorder as the first manifestation of CADASIL

    PubMed Central

    2014-01-01

    Background Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebrovascular disease, clinically characterized by variable manifestations of migraine, recurrent transient ischemic attack or lacunar strokes, cognitive decline, and mood disturbances. However, manic episodes have rarely been documented as an initial symptom of CADASIL and bipolar disorder presenting as the first manifestation in CADASIL has not been reported previously from evaluations by psychiatrists or psychological testing by psychologists. Case presentation A 53 year old woman developed symptoms of mania in her 50s leading to a personality change involving a continuously labile mood and irritability over a number of years. Neuropsychological testing revealed an intact memory, but impairment in attention and executive function. In the Rorschach test, she showed a high level of cognitive rigidity. Magnetic resonance imaging findings were very consistent with a diagnosis of CADASIL, which was confirmed by genetic testing for NOTCH3 mutations. Atypical antipsychotics proved to be helpful in treating her manic symptoms and for behavior control. Conclusion We present a novel case of CADASIL that first presented as bipolar disorder. We contend that when patients show a late onset personality change or chronically irritable mood that deteriorates over many years, an organic cause such as CADASIL must be considered. Further studies are needed to better understand the exact impacts of cerebral tissue lesions and psychiatric symptoms in CADASIL patients. PMID:24929957

  10. Rare Bone Diseases and Their Dental, Oral, and Craniofacial Manifestations

    PubMed Central

    Foster, B.L.; Ramnitz, M.S.; Gafni, R.I.; Burke, A.B.; Boyce, A.M.; Lee, J.S.; Wright, J.T.; Akintoye, S.O.; Somerman, M.J.; Collins, M.T.

    2014-01-01

    Hereditary diseases affecting the skeleton are heterogeneous in etiology and severity. Though many of these conditions are individually rare, the total number of people affected is great. These disorders often include dental-oral-craniofacial (DOC) manifestations, but the combination of the rarity and lack of in-depth reporting often limit our understanding and ability to diagnose and treat affected individuals. In this review, we focus on dental, oral, and craniofacial manifestations of rare bone diseases. Discussed are defects in 4 key physiologic processes in bone/tooth formation that serve as models for the understanding of other diseases in the skeleton and DOC complex: progenitor cell differentiation (fibrous dysplasia), extracellular matrix production (osteogenesis imperfecta), mineralization (familial tumoral calcinosis/hyperostosis hyperphosphatemia syndrome, hypophosphatemic rickets, and hypophosphatasia), and bone resorption (Gorham-Stout disease). For each condition, we highlight causative mutations (when known), etiopathology in the skeleton and DOC complex, and treatments. By understanding how these 4 foci are subverted to cause disease, we aim to improve the identification of genetic, molecular, and/or biologic causes, diagnoses, and treatment of these and other rare bone conditions that may share underlying mechanisms of disease. PMID:24700690

  11. Cervical spine manifestations in patients with inflammatory arthritides.

    PubMed

    Cha, Thomas D; An, Howard S

    2013-07-01

    The cervical spine can frequently become involved in patients with rheumatologic disorders, as a result of either the rheumatologic disease itself or age-associated degenerative processes that can also occur in the rest of the population. Awareness of the increased risk of cervical spine manifestations in patients with rheumatologic disorders enables early recognition and initiation of the appropriate treatment regimen. For example, patients with rheumatoid arthritis (RA) often have spinal instability which, if left untreated, can lead to neurological deficits. Biologic agents are effective in slowing the progression of the skeletal abnormality as well as for treating the RA, and this approach is often sufficient. However, early surgical intervention is recommended for patients with RA who develop neurologic deficits, as conservative approaches have limited effectiveness in this group. Spinal stability should be the primary surgical objective. For patients with ankylosing spondylitis, cervical spine surgery might be required either for fracture repair or to correct severe kyphosis. Understanding each condition's specific cervical spine manifestation and its natural history can help to clarify the appropriate indications for and timing of surgery to maximize patients' outcomes and limit complications. PMID:23528639

  12. T Helper Cell Subsets in Clinical Manifestations of Psoriasis.

    PubMed

    Diani, Marco; Altomare, Gianfranco; Reali, Eva

    2016-01-01

    Psoriasis is a chronic inflammatory skin disease, which is associated with systemic inflammation and comorbidities, such as psoriatic arthritis and cardiovascular diseases. The autoimmune nature of psoriasis has been established only recently, conferring a central role to epidermal CD8 T cells recognizing self-epitopes in the initial phase of the disease. Different subsets of helper cells have also been reported as key players in the psoriasis pathogenesis. Here, we reviewed the knowledge on the role of each subset in the psoriatic cascade and in the different clinical manifestations of the disease. We will discuss the role of Th1 and Th17 cells in the initiation and in the amplification phase of cutaneous inflammation. Moreover, we will discuss the recently proposed role of tissue resident Th22 cells in disease memory in sites of recurrent psoriasis and the possible involvement of Th9 cells. Finally, we will discuss the hypothesis of a link between T helper cell subsets recirculating from the skin and the systemic manifestations of psoriasis. PMID:27595115

  13. Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity.

    PubMed

    Kampylafka, Eleni I; Alexopoulos, Harry; Dalakas, Marinos C; Tzioufas, Athanasios G

    2016-01-01

    Neurological involvement is relatively common in the majority of systemic autoimmune diseases and may lead to severe morbidity and mortality, if not promptly treated. Treatment options vary greatly, depending on the underlying systemic pathophysiology and the associated neurological symptoms. Selecting the appropriate therapeutic scheme is further complicated by the lack of definite therapeutic guidelines, the necessity to differentiate primary neurological syndromes from those related to the underlying systemic disease, and to sort out adverse neurological manifestations caused by immunosuppressants or the biological agents used to treat the primary disease. Immunotherapy is a sine qua non for treating most, if not all, neurological conditions presenting in the context of systemic autoimmunity. Specific agents include classical immune modulators such as corticosteroids, cyclophosphamide, intravenous immunoglobulin, and plasma exchange, as well as numerous biological therapies, for example anti-tumor necrosis factor agents and monoclonal antibodies that target various immune pathways such as B cells, cytokines, and co-stimulatory molecules. However, experience regarding the use of these agents in neurological complications of systemic diseases is mainly empirical or based on small uncontrolled studies and case series. The aim of this review is to present the state-of-the-art therapies applied in various neurological manifestations encountered in the context of systemic autoimmune diseases; evaluate all treatment options on the basis of existing guidelines; and compliment these data with our personal experience derived from a large number of patients.

  14. Social Comparison Manifests in Event-related Potentials.

    PubMed

    Luo, Yi; Feng, Chunliang; Wu, Tingting; Broster, Lucas S; Cai, Huajian; Gu, Ruolei; Luo, Yue-jia

    2015-01-01

    Social comparison, a widespread phenomenon in human society, has been found to affect outcome evaluation. The need to belong to a social group may result in distinct neural responses to diverse social comparison outcomes. To extend previous studies by examining how social comparison with hierarchical characteristics is temporally processed, electroencephalography responses were recorded in the current study. Participants played a lottery game with two pseudo-players simultaneously and received both their own and the other two players' outcomes. Results of three event-related potential components, including the P2, the feedback-related negativity (FRN), and the late positive component (LPC), indicate that social comparison manifests in three stages. First, outcomes indicating a different performance from others elicited a larger P2 than evenness. Second, the FRN showed hierarchical sensitivity to social comparison outcomes. This effect manifested asymmetrically. Finally, large difference between the participant's outcome and the other two players' evoked a larger LPC than the medium difference and the even condition. We suggest that during social comparison, people detect if there is any difference between self and others, and then evaluate the information of this difference hierarchically, and finally interpret the situations in which oneself deviates from the group as most motivationally salient. PMID:26183734

  15. Antibody induction of lupus-like neuropsychiatric manifestations.

    PubMed

    Lawrence, David A; Bolivar, Valerie J; Hudson, Chad A; Mondal, Tapan K; Pabello, Nina G

    2007-01-01

    Although systemic lupus erythematosus (SLE) is usually evaluated with regard to autoimmune reactivity toward the kidney, there are multiple psychiatric abnormalities associated with this autoimmune disease. Lupus-prone male NZM88 mice, derived from NZB/NZW F1 mice, develop early neuropsychiatric manifestations without any signs of nephritis. In addition to the usual repertoire of antibody specificities, including autoantibodies to dsDNA and renal antigens, mice of this inbred strain express autoantibodies to numerous brain antigens. Here, we show that autoantibodies to brain antigens, assessed by Western analysis, are as individually varied as are the diverse neuropsychiatric manifestations observed in SLE patients. Additionally, a monoclonal antibody derived from the spleen of an untreated NZM88 male when injected into healthy BALB/cByJ, but not C57BL/6J, mice induced behaviors similar to those of lupus-prone NZM88 mice. This monoclonal antibody, which is specific to dynamin-1, binds preferentially in BALB/cByJ cortex and induces substantial expression of cytokines mainly in the hypothalamus. Thus, an antibody to just one brain antigen can induce multiple behavioral changes, and multiple autoantibodies to different brain antigens exist in lupus-prone mice; however, susceptibility to the induction of neurobehavioral deficits is dependent on host genetics.

  16. Dengue infection presenting with central nervous system manifestation.

    PubMed

    Kankirawatana, P; Chokephaibulkit, K; Puthavathana, P; Yoksan, S; Apintanapong, S; Pongthapisit, V

    2000-08-01

    The objective of this study was to investigate the possibility of dengue virus infection causing an abnormal neurologic presentation. Between 1996 and 1998, all pediatric patients with clinical manifestations of encephalitis-like illness who were admitted to the Department of Pediatrics, Siriraj Hospital were prospectively studied for any evidence of dengue virus infection. The diagnosis of dengue virus infection was based on mosquito viral isolation and serologic and polymerase chain reaction (PCR) evidence. Of 44 patients with the preliminary diagnosis of acute viral encephalitis, 8 were diagnosed with dengue infection. All of these 8 patients were diagnosed by serology. In addition to the serologic diagnosis, four also had positive PCR, one had positive viral isolation, and one had both positive PCR and viral isolation. Only two patients were diagnosed by serologic evidence alone. All except one had clinical courses and laboratory findings compatible with typical dengue infection. All had obvious encephalitic clinical manifestations with normal cerebrospinal fluid findings except one patient, who had mildly increased cerebrospinal fluid protein. All of these patients recovered completely and had benign clinical courses except one patient, who developed leakage symptoms. None had liver failure. Dengue virus can cause acute encephalopathy with fever. It can masquerade as other types of acute viral encephalitis. However, its clinical course and prognosis are usually favorable.

  17. Manifest and Subtle Cyclic Behavior in Nonequilibrium Steady States

    NASA Astrophysics Data System (ADS)

    Zia, R. K. P.; Weiss, Jeffrey B.; Mandal, Dibyendu; Fox-Kemper, Baylor

    2016-09-01

    Many interesting phenomena in nature are described by stochastic processes with irreversible dynamics. To model these phenomena, we focus on a master equation or a Fokker-Planck equation with rates which violate detailed balance. When the system settles in a stationary state, it will be a nonequilibrium steady state (NESS), with time independent probability distribution as well as persistent probability current loops. The observable consequences of the latter are explored. In particular, cyclic behavior of some form must be present: some are prominent and manifest, while others are more obscure and subtle. We present a theoretical framework to analyze such properties, introducing the notion of “probability angular momentum” and its distribution. Using several examples, we illustrate the manifest and subtle categories and how best to distinguish between them. These techniques can be applied to reveal the NESS nature of a wide range of systems in a large variety of areas. We illustrate with one application: variability of ocean heat content in our climate system.

  18. Amount of manifested test anxiety in the heterogeneous classroom.

    PubMed

    Gjesme, T

    1982-03-01

    The influence of the achievement motives, the future time orientation (FTO), the level of ability, sex of S, and perceived intrinsic instrumentality of school activity (PiI) were considered in relation to manifested test anxiety (i.e., state anxiety) at school. The analyses of results, based on data from 507 pupils in the sixth grade in elementary school showed that the achievement motives and the individual's FTO played the major roles, with success-oriented pupils having lower test anxiety than failure-oriented pupils, and high FTO pupils lower test anxiety than low FTO pupils. Ability was negatively related to test anxiety, and girls had higher test anxiety than boys. The PiI revealed no main effect on test anxiety. However, its significant interaction with the other variables indicated that the influence of the personality factors on test anxiety was most pronounced under high PiI condition. A number of interactions between the variables indicated that they had a complex relation to manifested test anxiety at school. PMID:7069636

  19. Managing Inflammatory Manifestations in Patients with Chronic Granulomatous Disease.

    PubMed

    Magnani, Alessandra; Mahlaoui, Nizar

    2016-10-01

    Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by lack of phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase, which results in inflammatory dysregulation and increased susceptibility to infections. Patients with CGD may develop severe obstructive disorders of the digestive tract as a result of their dysregulated inflammatory response. Despite a growing focus on inflammatory manifestations in CGD, the literature data on obstructive complications are far less extensive than those on infectious complications. Diagnosis and management of patients with concomitant predispositions to infections and hyperinflammation are particularly challenging. Although the inflammatory and granulomatous manifestations of CGD usually respond rapidly to steroid treatment, second-line therapies (immunosuppressants and biologics) may be required in refractory cases. Indeed, immunosuppressants (such as anti-tumor necrosis factor agents, thalidomide, and anakinra) have shown some efficacy, but the value of this approach is controversial, given the questionable risk-to-benefit ratio and the small numbers of patients treated to date. Significant progress in allogeneic hematopoietic stem cell transplantation (the only curative treatment for CGD) has been made through better supportive care and implementation of improved, reduced-intensity conditioning regimens. Gene therapy may eventually be an option for patients lacking a suitable donor; clinical trials with new, safer vectors are ongoing at a few centers. PMID:27299584

  20. Rare bone diseases and their dental, oral, and craniofacial manifestations.

    PubMed

    Foster, B L; Ramnitz, M S; Gafni, R I; Burke, A B; Boyce, A M; Lee, J S; Wright, J T; Akintoye, S O; Somerman, M J; Collins, M T

    2014-07-01

    Hereditary diseases affecting the skeleton are heterogeneous in etiology and severity. Though many of these conditions are individually rare, the total number of people affected is great. These disorders often include dental-oral-craniofacial (DOC) manifestations, but the combination of the rarity and lack of in-depth reporting often limit our understanding and ability to diagnose and treat affected individuals. In this review, we focus on dental, oral, and craniofacial manifestations of rare bone diseases. Discussed are defects in 4 key physiologic processes in bone/tooth formation that serve as models for the understanding of other diseases in the skeleton and DOC complex: progenitor cell differentiation (fibrous dysplasia), extracellular matrix production (osteogenesis imperfecta), mineralization (familial tumoral calcinosis/hyperostosis hyperphosphatemia syndrome, hypophosphatemic rickets, and hypophosphatasia), and bone resorption (Gorham-Stout disease). For each condition, we highlight causative mutations (when known), etiopathology in the skeleton and DOC complex, and treatments. By understanding how these 4 foci are subverted to cause disease, we aim to improve the identification of genetic, molecular, and/or biologic causes, diagnoses, and treatment of these and other rare bone conditions that may share underlying mechanisms of disease.

  1. Pulmonary manifestations of Sjögren syndrome, systemic lupus erythematosus, and mixed connective tissue disease.

    PubMed

    Mira-Avendano, Isabel C; Abril, Andy

    2015-05-01

    Interstitial lung disease is a common and often life-threatening manifestation of different connective tissue disorders, often affecting its overall prognosis. Systemic lupus erythematosus, Sjögren syndrome, and mixed connective tissue disease, although all unique diseases, can have lung manifestations as an important part of these conditions. This article reviews the different pulmonary manifestations seen in these 3 systemic rheumatologic conditions.

  2. Myopericarditis and severe myocardial dysfunction as the initial manifestation of systemic lupus erythematosus

    PubMed Central

    Peñataro, Joaquín S; De Mingo, Ana; Del Río, Ana; Martínez, José A; Heras, Magda

    2012-01-01

    Pericarditis is the most frequent cardiac manifestation of systemic lupus erythematosus (SLE). However, a large pericardial effusion as the initial manifestation of the disease is infrequent, especially when it is associated with myocardial damage. We describe an unusual case of a young female with pleuropericarditis and severe myocardial dysfunction as the initial manifestation of SLE. PMID:24062915

  3. 46 CFR 148.71 - Information included in the dangerous cargo manifest.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 46 Shipping 5 2013-10-01 2013-10-01 false Information included in the dangerous cargo manifest... § 148.71 Information included in the dangerous cargo manifest. The dangerous cargo manifest must include... the material is being transported; (e) The quantity of material loaded in each hold or...

  4. 46 CFR 148.71 - Information included in the dangerous cargo manifest.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 46 Shipping 5 2014-10-01 2014-10-01 false Information included in the dangerous cargo manifest... § 148.71 Information included in the dangerous cargo manifest. The dangerous cargo manifest must include... the material is being transported; (e) The quantity of material loaded in each hold or...

  5. 46 CFR 148.71 - Information included in the dangerous cargo manifest.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 46 Shipping 5 2012-10-01 2012-10-01 false Information included in the dangerous cargo manifest... § 148.71 Information included in the dangerous cargo manifest. The dangerous cargo manifest must include... the material is being transported; (e) The quantity of material loaded in each hold or...

  6. 46 CFR 148.71 - Information included in the dangerous cargo manifest.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 46 Shipping 5 2011-10-01 2011-10-01 false Information included in the dangerous cargo manifest... § 148.71 Information included in the dangerous cargo manifest. The dangerous cargo manifest must include... the material is being transported; (e) The quantity of material loaded in each hold or...

  7. 8 CFR 251.5 - Paper arrival and departure manifests for crew.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 8 Aliens and Nationality 1 2012-01-01 2012-01-01 false Paper arrival and departure manifests for... REGULATIONS ARRIVAL AND DEPARTURE MANIFESTS AND LISTS: SUPPORTING DOCUMENTS § 251.5 Paper arrival and... from the United States must submit arrival and departure manifests in a paper format in accordance...

  8. Recurrent diarrhea as a manifestation of temporal lobe epilepsy.

    PubMed

    Murai, Tomohiko; Tohyama, Teruhiko; Kinoshita, Masako

    2014-01-01

    A woman with temporal lobe epilepsy manifesting with repeated episodes of sudden diarrhea and loss of consciousness is reported. A 63-year-old, right-handed female presented with chief complaints of sudden diarrhea and loss of consciousness for almost three decades. The first attack occurred in her 30s, and similar attacks repeated several times in a year. Her attacks comprised abrupt abdominal discomfort, diarrhea, sudden emergence of old memories relating to when she had played with her brother in her childhood, and loss of consciousness during defecation. She had no convulsion or automatism and fully recovered in a few minutes. Every time she was transferred to emergency hospital by ambulance, she had examinations such as blood test, head computed tomography, electrocardiogram, abdominal ultrasound, and electroencephalography (EEG), but no specific diagnosis was made. On admission to our hospital, vital signs, neurological examination, and blood tests did not show abnormal findings. During long-term video-EEG monitoring for 40 h, she had no habitual event. Interictal EEG showed intermittent irregular delta waves and sharp regional transients in the left anterio-midtemporal area. Sharp transients were not as outstanding from background activities as to be defined as epileptiform discharges, but they were reproducible in morphology and distribution and appeared not only in sleep but also in wakefulness. Brain magnetic resonance imaging was unremarkable. Single-photon emission computed tomography showed a decrease of blood flow in the left frontal and temporal lobes. Wechsler Adult Intelligence Scale-III showed a decline of verbal comprehension. We concluded that the patient was suffering from partial epilepsy originating from the left temporal lobe. Carbamazepine markedly improved her seizures. Temporal lobe epilepsy can manifest with diverse autonomic symptoms and signs. Abdominal sensations often herald the onset of epileptic seizures. Among them is an uncommon

  9. An atlas of the morphological manifestations of hidradenitis suppurativa.

    PubMed

    Scheinfeld, Noah

    2014-04-16

    This article is dermatological atlas of the morphologic presentations of Hidradenitis Suppurativa (HS). It includes: superficial abscesses (boils, furnucles, carbuncles), abscesses that are subcutaneous and suprafascial, pyogenic granulomas, cysts, painful erythematous papules and plaques, folliculitis, open ulcerations, chronic sinuses, fistulas, sinus tracts, scrotal and genital lyphedema, dermal contractures, keloids (some that are still pitted with follicular ostia), scarring, skin tags, fibrosis, anal fissures, fistulas (i.e. circinate, linear, arcuate), scarring folliculitis of the buttocks (from mild to cigarette-like scarring), condyloma like lesions in intertrigous areas, fishmouth scars, acne inversa, honey-comb scarring, cribiform scarring, tombstone comedones, and morphia-like plaques. HS can co-exist with other follicular diseases such as pilonidal cysts, dissecting cellulitis, acne conglobata, pyoderma gangrenosum, and acanthosis nigricans. In sum, the variety of presentations of HS as shown by these images supports the supposition that HS is a reaction pattern.HS is a follicular based diseased and its manifestations involve a multitude of follicular pathologies [1,2]. It is also known as acne inversa (AI) because of one manifestation that involves the formation of open comedones on areas besides the face. It is as yet unclear why HS is so protean in its manifestations. HS severity is assessed using the Hurley Staging System (Table 1). It also remains unclear why hidradentitis may remain limited to Hurley Stage 1, evolve to the more confluent (Hurley Stage 2), or progress even further to the fully confluent (Hurley Stage 3).In addition, HS can be associated with other follicular based diseases such as pilonidal cysts (PCs) of the sacrum and buttocks, dissecting cellulitis (DC), and acne conglobata (AC), which usually involves the face, chest, When HS occurs with PCs, DC, and/or AC it is referred to as the follicular occlusion triad or tetrad [2]. HS

  10. Effect of maternal transmissions on clinical manifestations of myotonic dystrophy

    SciTech Connect

    Eguchi, I.; Koike, R.; Onodera, O.

    1994-09-01

    The mutation of myotonic dystrophy (DM) has been identified as unstable expansions of trinucleotide CTG repeat, located on chromosome 19q13-3. Although previous investigations have emphasized the strong association of the sizes of the CTG repeat with ages of onset as well as the clinical manifestations, effects of the paternal or maternal transmissions other than CTG repeats on the clinical manifestations in DM have not been evaluated in detail. To investigate how parental transmission affect the DM phenotype, we analyzed 15 cases of paternal transmission and 25 cases of maternal transmission. We have classified DM patients into 4 clinical grades. As in accordance with previous reports, there is a good correlation on sizes of the CTG repeat with their clinical features. The sizes of the CTG repeat in congenital DM patients (4.13{plus_minus}0.221 kbp) (Mean {plus_minus}SEM), who inherited mutant genes from their mothers, were not significantly larger than those of non-congenital DM patients (3.65 {plus_minus}0.36 kbp). As it has been well established that congenital DM patients are born to affected mothers, we investigated to see if there are any parental bias on the clinical manifestations in non-congenital DM. We classified each case into 4 classes depending on the size ranges of the CTG repeat (0 to 1.5 kbp, 1.5 to 3.0 kbp, 3.0 to 4.5 kbp, 4.5 kbp<). In each group of the size ranges of the CTG repeat, the distribution of cases among grades I to III were compared between paternally and maternally transmitted cases. There were statistically significant differences in the distributions of cases among grades I to III for the size ranges of 3 to 4.5 kbp expansions (p<0.01) and over 4.5 kbp expansions (p<0.05) on {chi}{sup 2} test, respectively. The results revealed that maternally transmitted cases tend to show severe phenotypes compared to paternally transmitted ones even if they have similar sizes of CTG repeat.

  11. An atlas of the morphological manifestations of hidradenitis suppurativa.

    PubMed

    Scheinfeld, Noah

    2014-01-01

    This article is dermatological atlas of the morphologic presentations of Hidradenitis Suppurativa (HS). It includes: superficial abscesses (boils, furnucles, carbuncles), abscesses that are subcutaneous and suprafascial, pyogenic granulomas, cysts, painful erythematous papules and plaques, folliculitis, open ulcerations, chronic sinuses, fistulas, sinus tracts, scrotal and genital lyphedema, dermal contractures, keloids (some that are still pitted with follicular ostia), scarring, skin tags, fibrosis, anal fissures, fistulas (i.e. circinate, linear, arcuate), scarring folliculitis of the buttocks (from mild to cigarette-like scarring), condyloma like lesions in intertrigous areas, fishmouth scars, acne inversa, honey-comb scarring, cribiform scarring, tombstone comedones, and morphia-like plaques. HS can co-exist with other follicular diseases such as pilonidal cysts, dissecting cellulitis, acne conglobata, pyoderma gangrenosum, and acanthosis nigricans. In sum, the variety of presentations of HS as shown by these images supports the supposition that HS is a reaction pattern.HS is a follicular based diseased and its manifestations involve a multitude of follicular pathologies [1,2]. It is also known as acne inversa (AI) because of one manifestation that involves the formation of open comedones on areas besides the face. It is as yet unclear why HS is so protean in its manifestations. HS severity is assessed using the Hurley Staging System (Table 1). It also remains unclear why hidradentitis may remain limited to Hurley Stage 1, evolve to the more confluent (Hurley Stage 2), or progress even further to the fully confluent (Hurley Stage 3).In addition, HS can be associated with other follicular based diseases such as pilonidal cysts (PCs) of the sacrum and buttocks, dissecting cellulitis (DC), and acne conglobata (AC), which usually involves the face, chest, When HS occurs with PCs, DC, and/or AC it is referred to as the follicular occlusion triad or tetrad [2]. HS

  12. [Occupational chronic arsenical poisoning. On the cutaneous manifestations (author's transl)].

    PubMed

    Hamada, T; Horiguchi, S

    1976-03-01

    A physical examination with detailed dermatological examination was made on 28 Japanese male workers, 22 to 63 years old, who had been exposed to arsenic for one to 24 years, in processes manufacturing lead arsenate, calcium arsenate and so on to be used as insecticides. Many corn-like punctate keratoses were seen on the palms and soles of 7 workers. The histopathological features in 6 specimens of punctate keratoses of palms and other regions of 4 workers were hyperkeratosis, acanthosis and elongation of rete ridge, but no malignant changes, although there were mild disorderly arrangements in part of stratum malpighii. Multiple lesions of sharply demarcated dullred patch with scale were found on the trunk and extremities of 3 workers. These lesions histopathologically showed the typical feature of Bowen's disease. Characteristic bronze pigmentation on the trunk and extremities was seen in 6 workers, and 5 of these 6 workers showed tiny normal or depigmented skin (raindrops) throughout the involved areas. These corn-like punctate keratoses mainly on the palms and soles, multiple Bowen's disease and diffuse bronze pigmentation with raindrop areas of hypopigmentation on the trunk and extremities were manifest almost in the same individuals, and it was concluded that they were characteristic cutaneous symptoms of chronic arsenical poisoning. There was no correlation between the intensity of cutaneous manifestations and the term of exposure to arsenic. Cutaneous manifestations due to arsenic were generally recognized as follows: diffuse pigmentation appears at first in one to two years after exposure to arsenic, then punctate hyperkeratotic lesions appear mainly on the palms and soles, and Bowen's lesions occur after several years or more. The use of arsenic has been abandoned in this factory since January, 1971. Diffuse bronze pigmentation now seems to be considerably lightened, as long time has passed after exposure to arsenic. However, since punctate hyperkeratotic

  13. [PAPILLOMAVIRUS INFECTION: PRINCIPLE CHARACTERISTICS, CLINICAL MANIFESTATIONS, VACCINE PROPHYLAXIS].

    PubMed

    Lopukhov, P D; Briko, N I; Khaldin, A A; Tsapkova, N N; Lupashko, O V

    2016-01-01

    Papillomaviruses are a large and diverse group of viruses. It includes approximately 200 fully described types that have been detected in humans. Human papilloma viruses (HPV) are etiologic agents during various, benign and malignant lesions of mucous membrane and skin epithelium. Very importantly, persistent HPV infection of certain types is a leading cause of carcinoma of uterine cervix, penis, vulva; vagina, anal canal and fauces (including tongue base and tonsils). HPV infection prophylaxis is the best means to control HPV-conditioned diseases, and vaccination, as had been demonstrated, --the most effective method of its prophylaxis. In this paper principle characteristics and clinical manifestations of papillomavirus infection, as well as effectiveness of vaccination against HPV are examined.

  14. Anophthalmia: an uncommon manifestation of neurofibromatosis type 1.

    PubMed

    Chen, Sheng; Pu, Jia-Li; Zhang, Jian-Min; Hong, Yuan

    2011-11-01

    Neurofibromatosis type 1 (NF-1) is an autosomal dominant, multisystem disorder, affecting approximately 1 of 3500 people. Ocular disorders, such as Lisch nodules, optic gliomas, and anterior segment defects, are typical with clinical presentation. Anophthalmia, as a rare eye malformation, has never been reported in patients with NF-1. We report a 27-year-old patient in whom clinical manifestations of café au lait spots, neurofibromas, osseous orbital dysplasia, and anophthalmia were observed. The diagnosis of NF-1 was made, according to clinical course and brain computed tomography and magnetic resonance imaging. Because the patient refused aggressive management approaches, she was managed conservatively and is well on follow-up. We suggest that patients presenting with anophthalmia need serious evaluation and that NF-1 needs to be considered in the differential diagnosis.

  15. Gallstone disease. The clinical manifestations of infectious stones.

    PubMed

    Smith, A L; Stewart, L; Fine, R; Pellegrini, C A; Way, L W

    1989-05-01

    Gallstones from 82 patients were examined under a scanning electron microscope for evidence of bacteria, and the findings were compared with the clinical manifestations of the disease. Bacteria were present in 68% of pigment stones and the pigment portions of 80% of composite stones. These gallstones were referred to as infectious stones. No bacteria were found in cholesterol gallstones. Acute cholangitis was diagnosed in 52% of patients with infectious stones and in 18% of patients with noninfectious stones. Over half of the patients with noninfectious stones presented with mild symptoms. Infectious stones were more often associated with a previous common duct exploration, an urgent operation, infected bile, a common duct procedure, and complications. These data show that gallstone disease is more virulent in patients whose gallstones contain bacteria.

  16. Pictorial review of intrathoracic manifestations of progressive systemic sclerosis

    PubMed Central

    AL-Jahdali, Hamdan; Rajiah, Prabhakar; Allen, Carolyn; Koteyar, Shyam Sunder; Khan, Ali Nawaz

    2014-01-01

    Intra-thoracic manifestations of progressive systemic sclerosis (PSS) are not well known particularly the imaging features, which forms the basis of accurate and timely diagnosis. The aim of this study is to familiarize the physicians and radiologists with these features. The diagnosis can remain elusive because of the non-specific nature of symptoms which mimic many common conditions. Thus, the diagnosis of PSS can be missed leading to continuous morbidity if the correct imaging is not pursued. The authors examined the records of rheumatology patient referrals of over a 5 year period. A hundred and seventy patients with systemic sclerosis and mixed connective tissue disorders were chosen for detailed study of the imaging available, which form the basis of this review. The images included conventional chest radiographs, digital radiographs computed radiography (CT) and high resolution computed tomography (HRCT). Where applicable computed pulmonary angiography (CTPA) and radionuclide scans were also interrogated. PMID:25276237

  17. Thrombosis in vasculitic disorders-clinical manifestations, pathogenesis and management.

    PubMed

    Katz, Ofrat Beyar; Brenner, Benjamin; Horowitz, Netanel A

    2015-09-01

    Inflammation and coagulation are known to affect each other in many ways. Vasculitis represents a group of disorders where blood vessels (small, medium, large or variable) are infiltrated with inflammatory cells. Accumulating evidence in the literature suggests both clinical and physiological association between vasculitis and thrombosis. Vasculitis-associated thrombosis involves arteries and veins, and a tight connection has been reported between the activity of vasculitis and the appearance of thrombosis. Pathophysiology of these relations is complex and not completely understood. While thrombophilic factors are associated with vasculitis, it remains unclear whether a true association with clinical thrombosis is present. Furthermore, several factors leading to hemostasis, endothelial injury and induction of microparticles were described as possibly accounting for thrombosis. Management of thrombosis in vasculitis patients is challenging and should be further assessed in randomized controlled studies. The current review describes clinical manifestations, pathogenesis and management of thrombosis associated with different vasculitides.

  18. Pyoderma Gangrenosum-A New Manifestation of Wilson Disease?

    PubMed

    Freg, George Naiem Ibrahiem; Shah, Venisha; Nagral, Aabha; Jhaveri, Ajay

    2016-03-01

    Seventeen year old girl, a known case of Wilson disease presented to us with a non-healing skin ulcer followed by appearance of jaundice, ascites and progressive fatigue of 1 month duration. She was diagnosed to have Wilson disease 5 years back and had been well controlled on d-penicillamine. On enquiry, she was found to be noncompliant with her medication in the preceding 6 months. On examination, she had severe pallor, icterus with moderate ascites and oedema feet. Investigations revealed severe haemolytic anemia and deranged liver function. The lesion was diagnosed to be pyoderma gangrenosum on skin biopsy. The appearance of a cutaneous lesion followed by deterioration in the liver disease and hemolysis suggested uncontrolled Wilson disease as the triggering factor. Chelation therapy improved her haemoglobin and liver function as well as led to healing of the ulcer. We describe pyoderma gangrenosum as a new manifestation of Wilson disease.

  19. Ocular manifestation of lymphoma in newly diagnosed cats.

    PubMed

    Nerschbach, V; Eule, J C; Eberle, N; Höinghaus, R; Betz, D

    2016-03-01

    Ocular manifestations of lymphoma are described in humans and dogs but rarely in cats. In this prospective study, cats with newly diagnosed and treatment-naïve lymphoma were evaluated concerning clinical stage and ophthalmologic findings. Twenty-six cats were included. In 12 cats (48%), ocular changes were documented. Uveitis anterior and posterior were predominant findings, being present in 58% of affected individuals. Other findings included exophthalmos, corneal surface lesions and chemosis. Eight cats received chemotherapy, two of which had ocular involvement. In these two cats, a complete remission of an anterior and a partial remission of a posterior uveitis were documented. Due to the detection of ocular involvement, a stage migration from stage IV to V occurred in four patients. In the light of these findings, an opthalmological examination may be considered as an important part of staging in feline lymphoma as well as of follow-up examination in affected cats.

  20. Oral manifestations of connective tissue disease and novel therapeutic approaches.

    PubMed

    Heath, Kenisha R; Rogers, Roy S; Fazel, Nasim

    2015-10-16

    Connective tissue diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and Sjögren syndrome (SS) have presented many difficulties both in their diagnosis and treatment. Known causes for this difficulty include uncertainty of disease etiology, the multitude of clinical presentations, the unpredictable disease course, and the variable cell types, soluble mediators, and tissue factors that are believed to play a role in the pathogenesis of connective tissue diseases. The characteristic oral findings seen with these specific connective tissue diseases may assist with more swift diagnostic capability. Additionally, the recent use of biologics may redefine the success rate in the treatment and management of the disease. In this review we describe the oral manifestations associated with SLE, SSc, and SS and review the novel biologic drugs used to treat these conditions.

  1. Oral manifestations of epidermolysis bullosa dystrophica: a rare genetic disease

    PubMed Central

    Parushetti, Anita Dundappa; Agrawal, Jiwanasha Manish; Nanjannawar, Lalita Girish; Agrawal, Manish Suresh

    2013-01-01

    Epidermolysis bullosa (EB) constitutes a group of phenotypically diverse genodermatoses, which manifests with blistering and erosions of the skin and mucous membranes as the unifying diagnostic feature. It is an acquired disease or inherited as either autosomal-dominant or recessive with an incidence of 1/50 000. The prominent clinical characteristic of the disease is the development of bullae or vesicles in mucosa or skin in response to minor trauma. It is a chronic mechanobullous disease characterised by auto antibodies against Type VII collagen. This paper documents a case of a man diagnosed with dominant dystrophic EB; describing the measures that dentists and healthcare providers should adopt in order to provide a safe and effective dental treatment. Early prevention protocols for these patients have also been discussed. PMID:23349175

  2. [Hepatic haemangioma as sistemic manifestation of rheumatoid arthritis].

    PubMed

    Babaeva, A R; Kalinina, E V; Solodenkova, K S

    2015-01-01

    This article presents the clinical data regarding to the incidence of hepatic haemangioma in rheumatoid arthritis (RA) patients. Haemangioma of the liver has been diagnosed by ultrasonography dramatically oftener in RA than in OA: in 8 from 37 RA pts and neither in 120 osteoarthritis pts. Hepatic haemangioma was associated with long standing erosive RA, extra-articular RA manifestations, high levels of rheumatoid factor and ACCP. Moreover in RA pts with hepatic haemangioma we revealed significantly raised levels of pro-inflammatory cytokines, which can impact on the angiogenesis in RA. Obtained clinical data may contribute in the understanding of liver vessels involvement in RA and pose the role of systemic connective tissue lesion and immune inflammation in this process.

  3. [Serrapeptase-induced lung injury manifesting as acute eosiniphilic pneumonia].

    PubMed

    Sasaki, S; Kawanami, R; Motizuki, Y; Nakahara, Y; Kawamura, T; Tanaka, A; Watanabe, S

    2000-07-01

    An 84-year-old man was referred to our hospital because of fever, cough, and hemoptysis. The patient had acute respiratory failure (PaO2 < 40 mmHg) on admission, with diffuse interstitial infiltration and bilateral pleural effusion. The bronchoalveolar lavage fluid was bloody, and contained a high percentage of eosinophils (32%). A diagnosis of acute eosinophilic pneumonia was established, and the patient made a rapid recovery after corticosteroids were administered. When the DLST (drug lymphocyte stimulation test) was performed after the corticosteroid therapy was stopped, it was positive for serrapeptase, which had been prescribed for chronic cystitis for 3 months before the onset of the pneumonia. This was a case of drug (serrapeptase)-induced pneumonitis manifesting as acute eosinophilic pneumonia.

  4. Splenorenal Manifestations of Bartonella henselae Infection in a Pediatric Patient.

    PubMed

    Rising, Taylor; Fulton, Nicholas; Vasavada, Pauravi

    2016-01-01

    Bartonella henselae is a bacterium which can cause a wide range of clinical manifestations, ranging from fever of unknown origin to a potentially fatal endocarditis. We report a case of Bartonella henselae infection in a pediatric-aged patient following a scratch from a kitten. The patient initially presented with a prolonged fever of unknown origin which was unresponsive to antibiotic treatment. The patient was hospitalized with worsening fevers and night sweat. Subsequent ultrasound imaging demonstrated multiple hypoechoic foci within the spleen. A contrast-enhanced CT of the abdomen and pelvis was also obtained which showed hypoattenuating lesions in the spleen and bilateral kidneys. Bartonella henselae IgG and IgM titers were positive, consistent with an acute Bartonella henselae infection. The patient was discharged with a course of oral rifampin and trimethoprim-sulfamethoxazole, and all symptoms had resolved following two weeks of therapy. PMID:27127672

  5. [Dermato-mucosal manifestations of Behçet's disease].

    PubMed

    Francès, C

    1999-11-01

    Oral and genital aphthae are the main clinical dermatologic manifestations of Behçet's disease. They look like those that occur in other aphthosis. Cutaneous lesions include pseudofolliculitis, folliculitis, erythema nodosum-like lesions, Sweet's-like lesions and pyoderma gangrenosum-like lesions. Histologically, these lesions are frequently perivascular with proeminent infiltrates of neutrophils and/or lymphocytes. Hypersensibility to needle pricks is explored by the pathergy test which sensibility is highly variable depending on the countries. When there is no systemic lesions requiring oral corticosteroids or immunosuppressive therapy, colchicine, aspirine, or dapsone may be prescribed. Thalidomide is sometimes required if aphthosis is refractory to other treatments despite its neurotixic and teratogenic effects. PMID:10637669

  6. Acute scrotal pain: an uncommon manifestation of renal vein thrombosis.

    PubMed

    Jou, Yeong-Chin; Jong, Ing-Chin; Hsieh, Ying-Chen; Kang, Chun-Hsiung

    2014-03-01

    The clinical manifestation of renal vein thrombosis varies with the speed and degree of venous occlusion. Such patients may be asymptomatic, have minor nonspecific symptoms such as nausea or weakness, or have more specific symptoms such as upper abdominal pain, flank pain, or hematuria. Acute scrotal pain is a very uncommon clinical expression of renal vein thrombosis. Here, we report a case of membranous glomerulonephritis-induced renal vein thrombosis presented with the symptom of acute scrotal pain caused by thrombosis-induced varicocele. This case report suggests that renal vein thrombosis should be considered in the diagnosis of acute scrotal pain; it also emphasizes that an investigation of retroperitoneum should be performed for adult patients with the sudden onset of varicocele.

  7. The multiple electrocardiographic manifestations of ventricular repolarization memory.

    PubMed

    Chiale, Pablo A; Etcheverry, Daniel; Pastori, Julio D; Fernandez, Pablo A; Garro, Hugo A; González, Mario D; Elizari, Marcelo V

    2014-08-01

    T wave "memory" is a peculiar variety of cardiac remodeling caused by a transient change in the course of ventricular depolarization (due to ventricular pacing, rate-dependent intraventricular block, ventricular preexcitation or tachyarrhythmias with wide QRS complexes). It is usually manifested by inverted T waves that appears when normal ventricular activation is restored. This phenomenon is cumulative and occurs earlier if the ventricular myocardium has previously been exposed to the same conditioning stimuli. In this article the different conditions giving rise to "classical" T wave memory development are reviewed and also "another" type of T wave memory is described. It is also shown that cardiac memory may induce not only negative (pseudo-primary) T waves but also a reversal of primary and pseudoprimary T waves leading to "normalization" of ventricular repolarization. The knowledge of these dissimilar consequences of T wave memory is essential to assess the characteristics of ventricular repolarization.

  8. [Consequences of extrahepatic manifestations of hepatitis C viral infection (HCV)].

    PubMed

    Pawełczyk, Agnieszka

    2016-01-01

    The hepatitis C virus (HCV) is a primarily hepatotropic virus. However, numerous extrahepatic symptoms are observed in patients chronically infected with HCV, e.g. cryoglobulinemia, lymphoproliferative disorders, kidney diseases, disturbances of the central and peripheral nervous system, thyroid gland, pancreas, lymph nodes and pituitary gland, that develop at various times after the infection. Complex mechanisms underlie these processes, both molecular, related to direct effects of the virus on cells or tissues and indirect mechanisms, resulting from the response of the immune system to infection (via cytokines or oxidative stress), and from the antiviral treatment used. Understanding these mechanisms may contribute to the definition of new prognostic factors, important for the early diagnosis of the infection, which in turn may improve treatment efficacy. This paper is a review of the incidence of selected extrahepatic manifestations of HCV infection and their underlying pathogenetic mechanisms and risk factors. PMID:27117111

  9. Cavitary pulmonary lesions in systemic lupus erythematosus: an unusual manifestation.

    PubMed

    Dalili, Amir Reza; Lotfi, Reza; Mousavi, Seyedeh Maryam

    2014-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown pathogenesis. The frequency of SLE with cavitary lesion manifestation is very rare and is thought to be due to infection or pulmonary embolism. A 19-year-old female diagnosed with SLE complicated by lupus nephritis and cavitary pulmonary lesion is presented in this case report. Other diseases that can lead to such lesions were ruled out in the patient. The patient improved briefly after the initiation of immunosuppressive therapy, but was unresponsive to supportive treatment due to pneumothorax. Pneumothorax is caused by cavitary lesions and possibly bronchopleural fistulas - these later caused respiratory distress and death. The patient did not show any improvement in the lesions after the initiation of immunosuppressive therapy. This case report suggests that the differential diagnosis of cavitary lung lesions should include SLE. PMID:25763160

  10. Thrombotic manifestations in SAPHO syndrome. Review of the literature.

    PubMed

    Carranco-Medina, Tatiana Elizabeth; Hidalgo-Calleja, Cristina; Calero-Paniagua, Ismael; Sánchez-González, María Dolores; Quesada-Moreno, Alba; Usategui-Martín, Ricardo; Pérez-Garrido, Laura; Gómez-Castro, Susana; Montilla-Morales, Carlos Alberto; Martínez-González, Olga; Del Pino-Montes, Javier

    2015-01-01

    SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a cluster of osteo-cutaneous manifestations that can lead to serious complications such as thrombosis of the subclavian vein or superior vena cava, mainly in patients with significant inflammatory involvement of the anterior-chest-wall. The objective of this study was to review the cases published in the medical literature related with the presence of thrombotic complications in patients diagnosed with SAPHO syndrome and to try to determine their possible pathogenic mechanism and risk factors. We analyzed 11 published reports of isolated clinical cases or case series, a total of 144 patients, which described a total of 15 cases of venous thrombosis. The clinical characteristics of these patients, evaluated to determine whether they meet the ASAS criteria for axial and peripheral spondyloarthritis, is analyzed the need for early diagnosis and treatment is highlighted.

  11. Triple A syndrome with ophthalmic manifestations in two siblings.

    PubMed

    Babu, Kalpana; Murthy, Krishna R; Babu, Narendra; Ramesh, S

    2007-01-01

    Triple A syndrome (Allgrove syndrome) is a rare, autosomal recessive disorder characterized by adrenocorticotropic hormone resistant adrenal insufficiency, alacrima, achalasia of the esophageal cardia, progressive neurological degeneration and occasionally autonomic instability. We report the ophthalmic manifestations in 2 siblings from a consanguineous family with this syndrome. A routine ophthalmic examination showed absence of palpebral portion of lacrimal gland, dry eyes and sluggish pupillary reflexes in both eyes of these patients. Both of these patients had achalasia cardia while the boy additionally had increased serum cortisol levels. Topical ocular lubricants were prescribed for both of them and Heller's cardiomyotomy with fundus plication was done for achalasia. These cases would create an awareness of this rare condition in ophthalmology in addition to suggesting its management. PMID:17595484

  12. Splenorenal Manifestations of Bartonella henselae Infection in a Pediatric Patient

    PubMed Central

    Rising, Taylor; Fulton, Nicholas; Vasavada, Pauravi

    2016-01-01

    Bartonella henselae is a bacterium which can cause a wide range of clinical manifestations, ranging from fever of unknown origin to a potentially fatal endocarditis. We report a case of Bartonella henselae infection in a pediatric-aged patient following a scratch from a kitten. The patient initially presented with a prolonged fever of unknown origin which was unresponsive to antibiotic treatment. The patient was hospitalized with worsening fevers and night sweat. Subsequent ultrasound imaging demonstrated multiple hypoechoic foci within the spleen. A contrast-enhanced CT of the abdomen and pelvis was also obtained which showed hypoattenuating lesions in the spleen and bilateral kidneys. Bartonella henselae IgG and IgM titers were positive, consistent with an acute Bartonella henselae infection. The patient was discharged with a course of oral rifampin and trimethoprim-sulfamethoxazole, and all symptoms had resolved following two weeks of therapy. PMID:27127672

  13. Neuropsychiatric manifestations in late-onset urea cycle disorder patients.

    PubMed

    Serrano, Mercedes; Martins, Cecilia; Pérez-Dueñas, Belén; Gómez-López, Lilian; Murgui, Empar; Fons, Carmen; García-Cazorla, Angels; Artuch, Rafael; Jara, Fernando; Arranz, José A; Häberle, Johannes; Briones, Paz; Campistol, Jaume; Pineda, Mercedes; Vilaseca, Maria A

    2010-03-01

    Inherited urea cycle disorders represent one of the most common groups of inborn errors of metabolism. Late-onset urea cycle disorders caused by partial enzyme deficiencies may present with unexpected clinical phenotypes. We report 9 patients followed up in our hospital presenting late-onset urea cycle disorders who initially manifested neuropsychiatric/neurodevelopmental symptoms (the most prevalent neuropsychiatric/neurodevelopmental diagnoses were mental retardation, attention-deficit hyperactivity disorder [ADHD], language disorder, and delirium). Generally, these clinical pictures did not benefit from pharmacological treatment. Conversely, dietary treatment improved the symptoms. Regarding biochemical data, 2 patients showed normal ammonium but high glutamine levels. This study highlights the fact that neuropsychiatric/neurodevelopmental findings are common among the initial symptomatology of late-onset urea cycle disorders. The authors recommend that unexplained or nonresponsive neuropsychiatric/neurodevelopmental symptoms appearing during childhood or adolescence be followed by a study of ammonia and amino acid plasmatic levels to rule out a urea cycle disorder.

  14. Obesity and heart failure: epidemiology, pathophysiology, clinical manifestations, and management.

    PubMed

    Alpert, Martin A; Lavie, Carl J; Agrawal, Harsh; Aggarwal, Kul B; Kumar, Senthil A

    2014-10-01

    Obesity is a risk factor for heart failure (HF) in both men and women. The mortality risk of overweight and class I and II obese adults with HF is lower than that of normal weight or underweight adults with HF of comparable severity, a phenomenon referred to as the obesity paradox. Severe obesity produces hemodynamic alterations that predispose to changes in cardiac morphology and ventricular function, which may lead to the development of HF. The presence of systemic hypertension, sleep apnea, and hypoventilation, comorbidities that occur commonly with severe obesity, may contribute to HF in such patients. The resultant syndrome is known as obesity cardiomyopathy. Substantial weight loss in severely obese persons is capable of reversing most obesity-related abnormalities of cardiac performance and morphology and improving the clinical manifestations of obesity cardiomyopathy.

  15. Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma

    PubMed Central

    Lee, E; Maselli, R; Ellis, W; Agius, M

    1998-01-01

    Morvan's fibrillary chorea is a rare disease characterised by symptoms which include neuromyotonia, cramping, weakness, pruritis, hyperhidrosis, insomnia, and delirium. The first case of Morvan's fibrillary chorea to be associated with clinical manifestations of myasthenia gravis with thymoma, psoriasis, and atopic dermatitis is reported. Muscle histopathology disclosed chronic denervation and myopathic changes and in vitro electrophysiology demonstrated both presynaptic and postsynaptic defects in neuromuscular transmission. Serum antibodies to acetylcholine receptors, titin, N-type calcium channels, and voltage gated potassium channels were detected. Plasmapheresis, thymectomy, and long term immunosuppression induced a dramatic resolution of symptoms. The association of thymoma with other autoimmune disorders and autoantibodies, and prolonged and sustained remission with chronic immunosuppression, place Morvan's fibrillary chorea on the range of neurological diseases arising as a paraneoplastic complication of cortical thymomas.

 PMID:9854961

  16. Borderline manifestations in the Rorschachs of male transsexuals.

    PubMed

    Murray, J F

    1985-10-01

    It was hypothesized that male transsexuals manifest a character structure consistent with Kernberg's criteria for borderline personality organization. Exploring this hypothesis, Kernberg's criteria for borderline personality organization were operationalized using Rorschach measures. The following variables were examined: aggression (Holt System Aggressive Content Section), object relations (Urist's Mutuality of Autonomy Scale), reality testing (Exner System X + %), and self/object differentiation (Exner System Special Scorings). A group of male college students, a group of male borderlines, and a group of male transsexuals were compared on the above variables. Compared to the normals, the transsexuals and borderlines displayed significantly more intense levels of aggression, a lower level of object relations, poorer reality testing, and impaired boundary differentiation. The transsexuals and borderlines did not differ significantly. The results were taken as suggesting that male gender dysphorics may be a sub-group of the wider borderline diagnostic category.

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

    PubMed

    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.

  18. Acute necrotizing pancreatitis as first manifestation of primary hyperparathyroidism

    PubMed Central

    Lenz, Jeroen I; Jacobs, Jimmy M; Op de Beeck, Bart; Huyghe, Ivan A; Pelckmans, Paul A; Moreels, Tom G

    2010-01-01

    We report the case of a female patient with severe acute necrotizing pancreatitis associated with hypercalcemia as first manifestation of primary hyperparathyroidism caused by a benign parathyroid adenoma. Initially the acute pancreatitis was treated conservatively. The patient subsequently underwent surgical resection of the parathyroid adenoma and surgical clearance of a large infected pancreatic pseudocyst. Although the association of parathyroid adenoma-induced hypercalcemia and acute pancreatitis is a known medical entity, it is very uncommon. The pathophysiology of hypercalcemia-induced acute pancreatitis is therefore not well known, although some mechanisms have been proposed. It is important to treat the provoking factor. Therefore, the cause of hypercalcemia should be identified early. Surgical resection of the parathyroid adenoma is the ultimate therapy. PMID:20556845

  19. Severe Psychotic Disorder as the Main Manifestation of Adrenal Insufficiency

    PubMed Central

    Farah, Julia de Lima; Lauand, Carolina Villar; Chequi, Lucas; Fortunato, Enrico; Pasqualino, Felipe; Bignotto, Luis Henrique; Batista, Rafael Loch; Aprahamian, Ivan

    2015-01-01

    We describe a case of severe psychotic disorder as the only manifestation of primary adrenal insufficiency. A 63-year-old man presented with psychotic symptoms without any prior psychiatric history. During the clinical and laboratorial investigation, exams revealed a normovolemic hyponatremia. The patient showed no other clinical signs or symptoms compatible with adrenal insufficiency but displayed very high ACTH and low serum cortisol concentrations. Brain magnetic resonance imaging showed no significant changes, including the pituitary gland. The patient was initially treated with intravenous corticosteroids, resulting in rapid remission of the psychotic symptoms. The association between adrenal insufficiency and neuropsychiatric symptoms is rare but these symptoms can often be the first clinical presentation of the disease. PMID:25954562

  20. Reversible dementia as a presenting manifestation of racemose neurocysticercosis

    PubMed Central

    Sharma, Sudhir; Modi, Manish; Lal, Vivek; Prabhakar, Sudesh; Bhardwaj, Amit; Sehgal, Rakesh

    2013-01-01

    Racemose cysticercosis is a less frequent presentation of neurocysticercosis (NCC). It's presentation and management is quite different from cerebral parenchymal NCC. Diagnosis of racemose cysticercosis is based on the combination of clinical, epidemiologic, radiographic, and immunologic information. Compared with cysticercus cellulose, which most commonly presents as seizures, racemose NCC due to its extraaxial location presents with raised intracranial pressure and meningitis, and frequently requires neurosurgical intervention. Dementia as a sole presenting feature of NCC is rare. We report a case of racemose NCC with dementia as the presenting manifestation. The outcome of dementia patients with NCC seems favorable in most cases therefore a high index of suspicion for NCC should be kept especially in endemic areas. PMID:23661971

  1. Oral Manifestations of Inflammatory Bowel Diseases: Two Case Reports

    PubMed Central

    Pereira, Manoela Seadi; Munerato, Maria Cristina

    2016-01-01

    Inflammatory bowel diseases (IBD) are known as chronic inflammatory disorders of the digestive tract, represented mainly by Crohn’s disease (CD) and ulcerative colitis (UC). Among the main oral manifestations of IBD are cobblestoning of the oral mucosa, labial swellings with vertical fissures, pyostomatitis vegetans, angular cheilitis, perioral erythema, and glossitis. In this sense, understanding these nosological entities by dentists would help reach early and differential diagnosis. Thus, two case reports are presented and discussed based on theoretical references obtained by a literature review. The first case report refers to an adult patient whose IBD diagnosis was established after stomatological assessment. The second case was a patient with CD diagnosed in childhood with characteristic oral lesions. PMID:26864508

  2. [Dengue fever with hemorrhagic manifestations after a stay in Thailand].

    PubMed

    Krippner, R; Hanisch, G; Kretschmer, H

    1990-06-01

    On returning from a 24-day long stay in Thailand a 30-year-old German woman fell ill with high fever and head and limb aches. Soon after a confluent petechial exanthem was noted, as well as conjunctival and gastrointestinal bleedings. Biochemical findings (fall in haemoglobin concentration and haematocrit, leucopenia and thrombocytopenia, rise in lactate dehydrogenase) and a rise in Dengue antibody titre to 1 : 320 (on the 13th day of illness) confirmed the diagnosis of Dengue fever with haemorrhagic manifestations. This mosquito-transmitted viral disease, while so far only rarely reported in tourists, should be considered in the differential diagnosis of fever of undetermined origin in travellers to the tropics.

  3. [PAPILLOMAVIRUS INFECTION: PRINCIPLE CHARACTERISTICS, CLINICAL MANIFESTATIONS, VACCINE PROPHYLAXIS].

    PubMed

    Lopukhov, P D; Briko, N I; Khaldin, A A; Tsapkova, N N; Lupashko, O V

    2016-01-01

    Papillomaviruses are a large and diverse group of viruses. It includes approximately 200 fully described types that have been detected in humans. Human papilloma viruses (HPV) are etiologic agents during various, benign and malignant lesions of mucous membrane and skin epithelium. Very importantly, persistent HPV infection of certain types is a leading cause of carcinoma of uterine cervix, penis, vulva; vagina, anal canal and fauces (including tongue base and tonsils). HPV infection prophylaxis is the best means to control HPV-conditioned diseases, and vaccination, as had been demonstrated, --the most effective method of its prophylaxis. In this paper principle characteristics and clinical manifestations of papillomavirus infection, as well as effectiveness of vaccination against HPV are examined. PMID:27029121

  4. [Cutaneous and mucosal manifestations associated with cocaine use].

    PubMed

    Imbernón-Moya, Adrián; Chico, Ricardo; Aguilar-Martínez, Antonio

    2016-06-17

    Complications due to cocaine are a public health problem. The typical cutaneous disease is leukocytoclastic vasculitis and/or thrombotic vasculopathy affecting mainly the ears. No intense systemic involvement is usually present, but there may be several cutaneous, mucosal and systemic manifestations. Other findings associated as arthralgia, neutropaenia or agranulocytosis, low titer positive antinuclear antibodies, antiphospholipid antibody positivity and neutrophil cytoplasmic antibodies against multiple antigens help the diagnosis. This disease requires a clinical suspicion with a clinical history, a complete physical examination and a broad differential diagnosis for an early and correct diagnosis. The course is usually self-limited. In most cases the only treatment is to discontinue the use of cocaine associated with symptomatic treatment, no proven benefit of systemic corticosteroids.

  5. Infertility and miscarriage: common pathways in manifestation and management.

    PubMed

    Agenor, Angena; Bhattacharya, Sohinee

    2015-07-01

    The relationship between miscarriage and fertility is complex. While most healthcare settings treat miscarriage as a problem of subfertility in assisted reproduction units, others believe that miscarriage occurs in super-fertile women. Infertile women undergoing assisted reproduction are at a greater risk of having a miscarriage especially at an advanced age compared with women conceiving naturally. Aberrant expression of immunological factors and chromosomal abnormalities underlie both infertility and miscarriage. Common risk factors include increased maternal age, obesity, smoking, alcohol, pre-existing medical conditions and anatomical abnormalities of the reproductive system. Management pathways of both conditions may be similar with pre-implantation genetic testing and assisted reproductive technology used in both conditions. This paper discusses the synergies and differences between the two conditions in terms of their epidemiology, etiopathogenesis, risk factors and management strategies. The two conditions are related as degrees of severity of reproductive failure with common pathways in manifestation and management.

  6. Human papillomavirus infection: etiopathogenesis, molecular biology and clinical manifestations.

    PubMed

    Leto, Maria das Graças Pereira; Santos Júnior, Gildo Francisco Dos; Porro, Adriana Maria; Tomimori, Jane

    2011-01-01

    Human papillomavirus (HPV) is a DNA virus that presents tropism for epithelial cells, causing infections of the skin and mucous membranes. Replication of HPV occurs in the nuclei of squamous cells and its life cycle is directly related to the differentiation program of the host cell. To date, nearly 100 different types of HPV have been characterized and there is a large number of other types that have not been sequenced yet. Besides being responsible for benign lesions of the skin and mucous membranes, HPV is also involved in the development of various mucocutaneous tumors: Bowen's disease, non-melanoma skin cancers and genital carcinomas. This review discusses the characteristics of HPV, malignant and benign mucous and skin manifestations caused by HPV, besides the main methods of detection and typing of the virus.

  7. Gastrointestinal manifestations of food allergies in pediatric patients.

    PubMed

    Garcia-Careaga, Manuel; Kerner, John A

    2005-10-01

    Foods that account for 90% of allergic reactions in children are cow's milk protein, eggs, peanut, soy, tree nuts, fish, and wheat. Food allergy can manifest as urticaria/angioedema, anaphylaxis, atopic dermatitis, respiratory symptoms, or a gastrointestinal (GI) disorder. GI allergic manifestations can be classified as immunoglobulin E (IgE) mediated (immediate GI hypersensitivity and oral allergy syndrome); "mixed" GI allergy syndromes (involving some IgE components and some non-IgE or T-cell-mediated components) include eosinophilic esophagitis and eosinophilic gastroenteritis. Non-IgE-mediated or T-cell-mediated allergic GI disorders include dietary protein enteropathy, protein-induced enterocolitis, and proctitis. All these conditions share a common denominator: the response of the immune system to a specific protein leading to pathologic inflammatory changes in the GI tract. This immunological response can elicit symptoms such as diarrhea, vomiting, dysphagia, constipation, or GI blood loss, symptoms consistent with a GI disorder. The detection of food allergies can be accomplished by the use of radioallergosorbent (RAST) testing and skin prick tests in helping to assess the IgE-mediated disorders. Patch tests may help evaluate delayed hypersensitivity reactions. Treatment of GI allergic disorders ranges from strict dietary elimination of offending food(s), use of protein hydrolysates, and use of L-amino acid-based formula when protein hydrolysates fail. Treatment with topical (for eosinophilic esophagitis) or systemic steroids is used if all dietary measures are unsuccessful. Maternal breast feeding or the use from birth of hydrolysate formulas (extensive or partial hydrolysates) may be efficacious in the prevention of atopic disease in "high-risk" families (with at least 1 parent or sibling with a history of atopic disease).

  8. Pathogenesis and clinical manifestations of juvenile rheumatoid arthritis.

    PubMed

    Hahn, Youn-Soo; Kim, Joong-Gon

    2010-11-01

    Juvenile rheumatoid arthritis (JRA) is the most common rheumatic childhood disease; its onset is before 16 years of age and it persists for at least 6 weeks. JRA encompasses a heterogeneous group of diseases that is classified according to 3 major presentations: oligoarthritis, polyarthritis, and systemic onset diseases. These presentations may originate from the same or different causes that involve interaction with specific immunogenetic predispositions, and result in heterogeneous clinical manifestations. An arthritic joint exhibits cardinal signs of joint inflammation, such as swelling, pain, heat, and loss of function; any joint can be arthritic, but large joints are more frequently affected. Extra-articular manifestations include high fever, skin rash, serositis, and uveitis. The first 2 types of JRA are regarded as T helper 1 (Th1) cell-mediated inflammatory disorders, mainly based on the abundance of activated Th1 cells in the inflamed synovium and the pathogenetic role of proinflammatory cytokines that are mainly produced by Th1 cell-stimulated monocytes. In contrast, the pathogenesis of systemic onset disease differs from that of other types of JRA in several respects, including the lack of association with human leukocyte antigen type and the absence of autoantibodies or autoreactive T cells. Although the precise mechanism that leads to JRA remains unclear, proinflammatory cytokines are thought to be responsible for at least part of the clinical symptoms in all JRA types. The effectiveness of biologic therapy in blocking the action of these cytokines in JRA patients provides strong evidence that they play a fundamental role in JRA inflammation.

  9. Cutaneous Manifestations in HTLV-I Positive Blood Donors

    PubMed Central

    Yazdanpanah, Mohammad Javad; Maleki, Masoud; Joneidi, Nasaibe; Khalighi, Amir Reza; Azarpazhooh, Mahmoud Reza; Khajedaluee, Mohammad; Tehranian, Farahnaz; Shahabi, Majid; Esmaeil Khayami, Mohammad; Livani, Fatemeh

    2013-01-01

    Objective(s): Infection with the human T-cell lymphotrophic virus type-I (HTLV-I) is endemic in Mashhad, Iran. In our research we evaluated the relation between exposure to this infection and the occurrence of dermatologic manifestations. Materials and Methods: 100 blood donors, who were seropositive but asymptomatic for infection with HTLV-I, were selected as case group. They were identified by the Blood Transfusion Organization Mashhad via the ELISA test and documented by PCR. Another 100 blood donors, that were seronegative for HTLV-I via the ELISA test and who were matched to the case group for age, gender, and existence of systemic diseases, were considered as the controls. Dermatologic evaluations and skin biopsies were performed if deemed necessary, and the results were statistically analyzed. Results: 73% of the case and control groups were male, while 27% in each of these groups were female. The mean age in both groups was 40.96±11.94 years. The examination indicated that 58% of the case group and 37% of the control group had cutaneous manifestations (P<0.01). The most common diseases found in the case group were aphthous stomatitis, herpes labialis, and non-genital warts, while common diseases found in the control group were herpes labialis, aphthous stomatitis, and skin tag. The frequency of aphthous stomatitis, eczema, and non-genital warts in the case group were significantly more than the control group (P<0.05). Conclusion : Cutaneous diseases can be found more frequent in asymptomatic carriers of HTLV-I than those who are HTLV-I seronegative. The aphthous stomatitis, eczema, and non-genital warts are more prevalent in those infected by HTLV-I. PMID:24470876

  10. Neurological Manifestations of Acute Posterior Multifocal Placoid Pigment Epitheliopathy

    PubMed Central

    Alkhotani, Ashjan; Shirah, Bader

    2016-01-01

    Background and Purpose Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an immune-mediated chorioretinal disease that causes acute visual symptoms with characteristic ophthalmoscopic findings. Neurological complications are rarely reported in the literature. Here we report two new cases of APMPPE that presented with neurological manifestations, one of which was associated with peripheral neuropathy, which has not been described before. Methods A retrospective database review of all patients with a diagnosis of APMPPE was performed. Clinical, ophthalmological, and neurological data were analyzed, and only cases of APMPPE with neurological complications were included. A literature review of several databases was also performed, and previous case reports were reviewed and analyzed in detail. Results In total, 56 cases of APMPPE-associated neurological complications were included in the analyses: 54 from the literature and 2 from our own practice. The most common complication was cerebral vasculitis, which affected 28 patients (50%), followed by headaches in 15 patients (26.8%). The other complications include sixth-cranial-nerve palsy, transient hearing loss, meningoencephalitis, cavernous sinus thrombosis, and viral meningitis. Conclusions This report adds to the literature of a novel association of APMPPE with peripheral neuropathy, and comprehensively reviews the neurological manifestations of this disease. A high level of suspicion should be applied when dealing with a case of APMPPE. We recommend applying detailed clinical neurological examinations and magnetic resonance imaging to APMPPE patients, and then early steroid treatment if the examination is positive or even suspicious. Early treatment with steroids and long-term treatment with immunosuppressive azathioprine with interval neurological evaluations will contribute positively to the outcomes and avoid fatal complications, namely strokes.

  11. Castleman Disease: A Rare Condition with Endocrine Manifestations

    PubMed Central

    Correa, Ricardo

    2015-01-01

    Castleman disease (CD) most commonly affects lymphoid tissues in the thorax, abdomen, pelvis, and neck. Extralymphatic tissues, such as lacrimal glands, lung, pancreas, larynx, parotid, meninges, and even muscles, have also been reported as sites. The etiology is unknown and its incidence has not been reported in the literature. Castleman disease can be classified clinically into a unicentric or multicentric form, depending on the number of lymph nodes involved, and histologically into a hyaline vascular variant, plasma cell, mixed cellular, or plasmablastic variant. The disease has a predominantly inflammatory background, reflected in high levels of vascular endothelial growth factor (VEGF) and interleukin-6 (IL-6). The role of cytokines in CD explains the clinical presentation. The clinical scenario varies widely, based mainly on the histologic type. Unicentric CD usually presents without symptomatology, whereas multicentric manifests with fatigue, abdominal or thoracic pain, cytopenias, and/or B- symptoms (10% weight loss in the last six months, nocturnal diaphoresis, and fever). The endocrinopathy has a wide range of manifestations, affecting either the pituitary or other target organs. Achieving the diagnosis is complicated and there is no laboratory or imaging pathognomonic for this disease. The gold standard is an excisional biopsy from an affected lymph node. The treatment depends on the type of CD. Unicentric CD has a good response to excisional surgery. However, in multicentric CD (MCD), surgery may provide transient relief of symptoms but with a rebound effect, so it is not considered a good method. The use of chemotherapy, monoclonal antibodies, glucocorticoids, and thalidomide has shown some improvement in MCD.  PMID:26719823

  12. Clinical Manifestations among Children with Chronic Functional Constipation

    PubMed Central

    Dehghani, Seyed Mohsen; Kulouee, Niloofar; Honar, Naser; Imanieh, Mohammad-Hadi; Haghighat, Mahmood; Javaherizadeh, Hazhir

    2015-01-01

    BACKGROUND Constipation is one of the most frequent cause of patient visits to pediatric gastroenterology clinics. Early diagnosis and treatment is important. There are few studies about clinical manifestations of constipation in children. We aimed to find the relative frequency of gastrointestinal manifestations of constipation among constipated children. METHODS This cross-sectional study was carried out on children aged < 18 years old with chronic functional constipation referred to Imam Reza Clinic of Shiraz University of Medical Sciences. Children with organic causes of chronic constipation were excluded from study. Rome III criteria were used for defining constipation. The duration of study was 1 year starting from September 2010. Abdominal pain, fecal mass, rectal bleeding, anorexia, fecal soiling, retentive posture, withholding behavior, anal fissure, and peri-anal erythema were recorded for each case based on history and physical examination. Data were analyzed using SPSS software, version 13.0 (Chicago, IL, USA). RESULTS Of 222 children with functional constipation, 124(55.9%) were girls and 98 (44.1%) were boys with a mean ± SD age of 5±3.12 years. The mean ± SD duration of constipation was 2.2±1.9 years. Large and hard stool was present in 93.7% of the patients. Painful defecation and withholding behavior were seen in 92.3% and 91.9% of the patients, respectively. Fecal impaction was more frequent among boys compared with girls (p<0.01). Fecal soiling was present in 40.8% of the boys and 28.2% of the girls (p=0.04). CONCLUSION Large and hard stool, painful defecation and withholding behavior were the most frequent signs or symptoms among children with chronic functional constipation. Fresh rectal bleeding and anal fissure were the least frequent signs and symptoms in this group. PMID:25628851

  13. A Rare Manifestation of Uveitis-glaucoma-hyphema Syndrome

    PubMed Central

    Leal, Inês; Faria, Mun Yueh; Pinto, Luís Abegão

    2016-01-01

    ABSTRACT Aims: To report a case of a patient who developed uveitis-glaucoma-hyphema (UGH) syndrome after an uneventful cataract surgery and to discuss risk factors, diagnostic challenges, management options, and clinical implications. Background: Uveitis-glaucoma-hyphema syndrome is a rare but potentially serious cataract surgery complication. Clinical manifestations include increased intraocular pressure (IOP), anterior chamber inflammation, and recurrent hyphema or microhyphema. Uveitis-glaucoma-hyphema Plus syndrome also includes accompanying vitreous hemorrhage. Although classically associated with rigid anterior chamber intraocular lenses (lOLs), cases of malpositioning and subluxated posterior chamber lOLs have also been described as possible triggers. Case description: We report a case of a 70-year-old Caucasian man who developed UGH Plus syndrome after an uneventful cataract surgery with an lOL implanted in the capsular bag. During postoperative follow-up, persistent intraocular inflammation, increased IOP, hyphema, and vitreous hemorrhage were consistent with this diagnosis. Slit-lamp examination demonstrated progressive localized iris atrophy, compatible with chafing of the posterior iris by the IOL haptic as the trigger for UGH syndrome. A pars plana vitrectomy was performed and a retropupillary intraocular lens was implanted. No further complications occurred during follow-up. Conclusion and clinical significance: Given the increasing prevalence of single-piece lOLs implanted in the capsular bag, it is important to recognize UGH syndrome as a rare but potentially serious complication. How to cite this article: Sousa DC, Leal I, Faria MY, Pinto LA. A Rare Manifestation of Uveitis-glaucoma-hyphema Syndrome. J Curr Glaucoma Pract 2016;10(2):76-78. PMID:27536051

  14. Manifestations of integrated public health policy in Dutch municipalities.

    PubMed

    Peters, Dorothee; Harting, Janneke; van Oers, Hans; Schuit, Jantine; de Vries, Nanne; Stronks, Karien

    2016-06-01

    Integrated public health policy (IPHP) aims at integrating health considerations into policies of other sectors. Since the limited empirical evidence available may hamper its further development, we systematically analysed empirical manifestations of IPHP, by placing policy strategies along a continuum of less-to-more policy integration, going from intersectoral action (IA) to healthy public policy (HPP) to health in all policies (HiAP). Our case study included 34 municipal projects of the Dutch Gezonde Slagkracht Programme (2009-15), which supports the development and implementation of IPHP on overweight, alcohol and drug abuse, and smoking. Our content analysis of project application forms and interviews with all project leaders used a framework approach involving the policy strategies and the following policy variables: initiator, actors, policy goals, determinants and policy instruments. Most projects showed a combination of policy strategies. However, manifestations of IPHP in overweight projects predominantly involved IA. More policy integration was apparent in alcohol/drugs projects (HPP) and in all-theme projects (HiAP). More policy integration was related to broad goal definitions, which allowed for the involvement of actors representing several policy sectors. This enabled the implementation of a mix of policy instruments. Determinants of health were not explicitly used as a starting point of the policy process. If a policy problem justifies policy integration beyond IA, it might be helpful to start from the determinants of health (epidemiological reality), systematically transform them into policy (policy reality) and set broad policy goals, since this gives actors from other sectors the opportunity to participate.

  15. Epidemiology of neurological manifestations in Sjögren's syndrome: data from the French ASSESS Cohort

    PubMed Central

    Carvajal Alegria, Guillermo; Guellec, Dewi; Mariette, Xavier; Gottenberg, Jacques-Eric; Dernis, Emmanuelle; Dubost, Jean-Jacques; Trouvin, Anne-Priscille; Hachulla, Eric; Larroche, Claire; Le Guern, Veronique; Cornec, Divi; Devauchelle-Pensec, Valérie; Saraux, Alain

    2016-01-01

    Objectives Neurological manifestations seem common in primary Sjögren's syndrome (pSS) but their reported prevalences vary. We investigated the prevalence and epidemiology of neurological manifestations in a French nationwide multicentre prospective cohort of patients with pSS, the Assessment of Systemic Signs and Evolution in Sjögren's syndrome (ASSESS) cohort. Methods The ASSESS cohort, established in 2006, includes 395 patients fulfilling American–European Consensus Group criteria for pSS. Demographic and clinical data were compared between patient groups with and without neurological manifestations, and across patient groups with peripheral nervous system (PNS) manifestations, central nervous system (CNS) manifestations and no neurological manifestations. Results Data at inclusion were available for 392 patients, whose mean age was 58±12 years. Mean follow-up was 33.9 months. Neurological manifestations were present in 74/392 (18.9%) patients, including 63 (16%) with PNS manifestations and 14 (3.6%) with CNS manifestations. Prevalences were 9.2% for pure sensory neuropathy, 5.3% for sensorimotor neuropathy, 1.3% for cerebral vasculitis and 1.0% for myelitis. Neurological manifestations were associated with greater pSS activity as assessed using the ESSDAI (9.4±6.8 vs 4.3±4.8; p<0.001) and proportion of patients taking immunomodulatory/immunosuppressive drugs (32.4% (24/74) versus 13.8% (44/318), p=0003). New neurological symptoms were more common in patients with than without prior neurological manifestations (RR=3.918 (95% CI 1.91 to 8.05); p<0.001). Conclusions Prevalences of peripheral and central neurological manifestations in pSS are about 15% and 5%, respectively. Neurological manifestations are associated with greater pSS activity. New neurological manifestations are more common in patients with prior neurological involvement. PMID:27110384

  16. Corneal lesion as the initial manifestation of tyrosinemia type II.

    PubMed

    Tsai, Chun-Pin; Lin, Pei-Yu; Lee, Ni-Chung; Niu, Dau-Ming; Lee, Shui-Mei; Hsu, Wen-Ming

    2006-06-01

    Tyrosinemia type II (Richner-Hanhart syndrome) is a rare autosomal recessive disease with deficiency of tyrosine aminotransferase and subsequently increasing level of serum tyrosine. We report the case of a 2-year-old girl who was referred due to bilateral corneal lesions. Slit-lamp examination showed small granular white deposits arranged in a dendritic pattern in the superficial central cornea of both eyes. Physical examination revealed painful, non-pruritic, hyperkeratotic plaques on the soles, palms and fingertips. Mental evaluation demonstrated developmental delay for her age. Blood examination revealed serum tyrosine level to be 1868 microM (normal range, 30-110 microM), which decreased to 838 microM with 2-month diet on tyrosine and phenylalanine restriction. The corneal and skin lesions resolved completely. However, the corneal deposits recurred a month later as her mother failed to strictly control the diet because the little girl was losing weight and activity. With specific formula and adjusted diet regimen, the corneal lesions decreased again. Corneal pseudodendritic deposits may be the initial manifestation in patients with tyrosinemia type II. Early diagnosis and intervention with diet control are crucial for preventing permanent visual and developmental deficits. Corneal deposits can be one of the parameters in monitoring the efficacy of diet control.

  17. Severe congenital thrombocytopaenia – first clinical manifestation of Noonan syndrome

    PubMed Central

    Nunes, Paula; Aguilar, Sara; Prado, Sara Noéme; Palaré, Maria João; Ferrão, Anabela; Morais, Anabela

    2012-01-01

    This report focuses on a male infant, the first born of non-consanguineous parents diagnosed with polyhydramnios at 26 weeks of gestation. The newborn was admitted during the neonatal period with bleeding diathesis associated with a low platelet count at birth (5×109/l).The authors registered a persistent low platelet count (9000–129 000/l) during the infants 1st year of life. Physical examination revealed a petechial rash, a dysmorphic face and bilateral cryptorchidism, in the absence of organomegaly. Additionally, cardiologic evaluation revealed an aortic valve dysplasia and an atrial septal defect, while bone marrow biopsy and aspiration were found normal. Throughout the investigation, the authors excluded congenital infection, alloimmune and familiar thrombocytopaenia, Fanconi anaemia and thrombocytopaenia absent radius syndrome. The cytogenetic analysis revealed a mutation in the PTPN11 gene associated with Noonan syndrome. Here the author highlights that severe neonatal thrombocytopaenia is a manifestation that should be considered in the diagnosis and clinical management of Noonan’s syndrome. PMID:22605701

  18. Superwarfarin intoxication: hematuria is a major clinical manifestation.

    PubMed

    Wu, Yi-Feng; Chang, Cheng-Shyong; Chung, Chih-Yuan; Lin, Hsuan-Yu; Wang, Chuan-Cheng; Shen, Ming-Ching

    2009-09-01

    Since superwarfarin is popular and readily available in stores, it may cause intoxication or overexposure, which can result in coagulopathy or abnormal bleeding in humans and, thus, is an important public health problem. We report our clinical experience with superwarfarin intoxication. Nine patients, including eight patients who had histories of ingesting superwarfarin, were studied. Of the patients, hematuria occurred in eight. Laboratory tests among the nine patients showed extremely prolonged prothrombin times and activated partial thromboplastin times, which could be corrected to normal by mixing 1:1 with normal pooled plasma; they also had very low functional levels of factor II, VII, IX, X, and proteins C and S, but normal functional levels of factors V, VIII, fibrinogen, and anti-thrombin III. Large doses of vitamin K1 were needed for 3 months or more to treat and correct the coagulopathy among the patients. The majority of the patients presented with gross hematuria, suggesting that hematuria is probably a major clinical manifestation of superwarfarin intoxication. Prolonged use of large doses of vitamin K1 is needed for the treatment of superwarfarin intoxication.

  19. Multisystem Radiologic Manifestations of Erdheim-Chester Disease

    PubMed Central

    Lodhi, Umairullah; Sarmast, Uzair; Khan, Saadullah; Yaddanapudi, Kavitha

    2016-01-01

    Erdheim-Chester Disease is a rare form of multiorgan non-Langerhans' cell histiocytosis that affects individuals between the ages of 50 and 70 with an equal distribution among males and females. It is associated with significant morbidity and mortality that is mostly due to infiltration of critical organs. Some of the sites that Erdheim-Chester Disease affects include the skeletal system, central nervous system, cardiovascular system, lungs, kidneys (retroperitoneum), and skin. The most common presenting symptom of Erdheim-Chester Disease is bone pain although a large majority of patients are diagnosed incidentally during a workup for a different disease process. Diagnosing Erdheim-Chester Disease is challenging due its rarity and mimicry to other infiltrative processes. Therefore, a multimodality diagnostic approach is employed with imaging being at the forefront. As of date, a comprehensive radiologic review of the manifestations of Erdheim-Chester Disease has rarely been reported. Here we present radiologic findings of an individual suffering from Erdheim-Chester Disease. PMID:27340583

  20. Skeletal manifestations of scurvy: a case report from dubai.

    PubMed

    Noordin, Shahryar; Baloch, Naveed; Salat, Muhammad Sohail; Rashid Memon, Abdul; Ahmad, Tashfeen

    2012-01-01

    Introduction. Nutritional deficiencies are rarely reported in developed countries. We report a child of Pakistani origin brought up in Dubai who developed skeletal manifestations of scurvy due to peculiar dietary habits. Case Presentation. A 4.5 year old boy presented with pain and swelling of multiple joints for three months and inability to walk for two months. Dietary history was significant for exclusive meat intake for the preceding two years. On examination the child's height and weight were below the 5th percentile for his age. He was pale and tachycardic. There was significant swelling and tenderness over the wrist, knee and ankle joints, along with painful restriction of motion. Basic blood workup was unremarkable except for anemia. However, X-rays showed delayed bone age, severe osteopenia of the long bones, epiphyseal separation, cortical thinning and dense zone of provisional calcification, suggesting a radiological diagnosis of scurvy. The child was started on vitamin C replacement therapy. Over the following two months, the pain and swelling substantially reduced and the child became able to walk. Repeat X-rays showed improvement in the bony abnormalities. Conclusion. Although scurvy is not a very commonly encountered entity in the modern era, inappropriate dietary intake can lead to skeletal abnormalities which may be confused with rickets. A high index of suspicion is thus required for prompt diagnosis of scurvy in patients with bone and joint symptoms.