Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Anatomic and functional neuroimaging with magnetic resonance imaging (MRI) includes the technology more widely known as magnetic resonance spectroscopy (MRS). Now a routine automated "add-on" to all clinical magnetic resonance scanners, MRS, which assays regional neurochemical health and disease, is therefore the most accessible diagnostic tool for clinical management of neurometabolic disorders. Furthermore, the noninvasive nature of this technique makes it an ideal tool for therapeutic monitoring of disease and neurotherapeutic decision making. Among the more than 100 brain disorders that fall within this broad category, MRS contributes decisively to clinical decision making in a smaller but growing number. In this review, we will cover how MRS provides therapeutic impact in brain tumors, metabolic disorders such as adrenoleukodystrophy and Canavan's disease, Alzheimer's disease, hypoxia, secondary to trauma or ischemia, human immunodeficiency virus dementia and lesions, as well as systemic disease such as hepatic and renal failure. Together, these eight indications for MRS apply to a majority of all cases seen. This review, which examines the role of MRS in enhancing routine neurological practice and treatment concludes: 1) there is added value from MRS where MRI is positive; 2) there is unique decision-making information in MRS when MRI is negative; and 3) MRS usefully informs decision making in neurotherapeutics. Additional efficacy studies could extend the range of this capability.
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PMID:Efficacy of proton magnetic resonance spectroscopy in neurological diagnosis and neurotherapeutic decision making. 1589 45

The relationship between oral and general health has been increasingly recognised during the past two decades. Several epidemiological studies have linked poor oral health with cardiovascular disease, poor glycaemic control in diabetics, low birth-weight pre-term babies, and a number of other conditions, including rheumatoid arthritis and osteoporosis. Oral infections are also recognised as a problem for individuals suffering from a range of chronic conditions, including cancer and infection with human immunodeficiency virus, as well as patients with ventilator-associated pneumonia. This review considers the systemic consequences of odontogenic infections and the possible mechanisms by which oral infection and inflammation can contribute to cardiovascular disease, as well as the oral conditions associated with medically compromised patients. A large number of clinical studies have established the clinical efficacy of topical antimicrobial agents, e.g., chlorhexidine and triclosan, in the prevention and control of oral disease, especially gingivitis and dental plaque. The possible risks of antimicrobial resistance are a concern, and the benefits of long-term use of triclosan require further evaluation. Oral infections have become an increasingly common risk-factor for systemic disease, which clinicians should take into account. Clinicians should increase their knowledge of oral diseases, and dentists must strengthen their understanding of general medicine, in order to avoid unnecessary risks for infection that originate in the mouth.
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PMID:Oral infections and systemic disease--an emerging problem in medicine. 1771 25

There currently is no animal model of JC virus-associated progressive multifocal leukoencephalopathy (PML). Reactivation of simian virus 40 (SV40) in immunosuppressed rhesus monkeys, however, rarely causes a PML-like illness. We sought to isolate a neurotropic clone of SV40 and determine its pathogenic potential in monkeys. The clone SV40CNS1 was amplified by polymerase chain reaction from the brain DNA of a simian/human immunodeficiency virus-infected monkey that had developed PML and meningoencephalitis. Compared with the SV40 prototype 776, SV40CNS1 had a small number of single-amino-acid mutations and caused a productive infection in monkey fibroblasts. It was inoculated into 2 SV40-negative, simian/human immunodeficiency virus-immunosuppressed monkeys. Both animals developed meningoencephalitis with productive SV40 infection of cerebral cortical neurons and glia in the superficial layers of the cortex and at the gray-white junction. Focal SV40-infected cells were also found in the cerebellar molecular and granule cell layers and white matter. Both animals also developed disseminated SV40 infection with nephritis and pneumonitis. Thus, SV40CNS1 is infectious and pathogenic in immunosuppressed monkeys, but it induces encephalitis with fulminant productive infection in cortical neurons and systemic disease, rather than PML. These findings shed new light on SV40 neurotropism and expand the host cell range of this virus.
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PMID:Productive simian virus 40 infection of neurons in immunosuppressed Rhesus monkeys. 1864 24

Chronic mucocutaneous candidiasis (CMC) is a rare disease associated with immunodeficiency and characterized by persistent and refractory infections of the skin, appendages and mucous membranes caused by members of the genus Candida. Several different disorders are classified under this common denominator, including chronic and recurrent mucocutaneous infections due to Candida spp., which are sometimes linked to autoimmune endocrinopathies. These fungal infections are usually confined to the mucocutaneous surface, with little propensity for systemic disease or septicemia. We describe a patient with CMC who had an esophageal candidiasis refractory to treatment for decades and who developed an epidermoid esophageal cancer. No risk factors such as familiar susceptibility, smoking, alcohol drinking, or living in an endemic area were verified. This case report suggests the participation of nitrosamine compounds produced by chronic Candida infections as a risk factor for esophageal cancer in a patient with autosomal-dominant chronic mucocutaneous candidiasis.
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PMID:Esophageal cancer associated with chronic mucocutaneous candidiasis. Could chronic candidiasis lead to esophageal cancer? 1879 15

The aim of this paper was to provide an update on necrotizing periodontal disease (NPD). Original papers on this subject published in English from January 2002 until December 2007 were located in MEDLINE/PubMed database. Other sources were taken from the references of the selected papers. The clinical features of NPD include necrosis and ulceration of the gingival margin and the interdental papilla. These signs are usually associated to halitosis, pain, spontaneous gingival bleeding, malaise and sometimes fever. NPD usually occurs in developing countries, but few cases have also been reported in industrialized nations. The predisposing factors associated with the onset and progression of NPD include immunodeficiency, malnutrition, stress, smoking and poor oral hygiene. The treatment of NPD is dependent on the presence of pain and gingival necrosis. Systemic antibiotic therapy is recommended when a systemic disease is suspected.
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PMID:[Necrotizing periodontal disease: an update]. 1904 50

Pyoderma gangrenosum is an uncommon chronic ulcerative dermatosis with unknown aetiology and with a pathology which is still obscure. In 15-45% of cases, it is related to intestinal chronic inflammatory disease (MICI), to a systemic disease that it can sometimes reveals or to an immunodeficiency This disease starts whether with a pustule, a bubble or a nodule which leads during its evolvement to a superficial ulceration with clear edges. Its diagnosis is easy and is mainly clinical. It is a disease which is localized preferably in lower limbs. Treatment is mainly based on oral route corticotherapy. We report a case of gangrenosum pyoderma localized on the penis in a 43-year-old HIV infected patient. It is an uncommon localization, misleading and delicate. We have treated successfully this patient by oral corticotherapy combined with local antiseptic treatments for 6 months.
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PMID:[An uncommon localization of pyoderma gangrenosum on the penis of an HIV infected patient in the Ivory Coast]. 1958 26

An important task for both dentists and pediatricians dealing with patients manifesting different oral lesions is to be able to differentiate changes signaling systemic disease from those appearing without any concomitant serious health problem. In this article, symptomatology of selected primary immune deficiency diseases are discussed with particular emphasis on oral manifestations reported in this group of disorders. Facial, dental, and oral findings compose a constellation of symptoms observed in immunodeficiency diseases. Predisposition to bacterial invasion, cytokine dysregulation, tissue inflammatory process, and necrosis lead to early-onset oral lesions and periodontitis. Developmental abnormalities, periodontal disease, and oral lesions may accompany immunodeficiency and require particular awareness directed toward diagnosis of an underlying disease of the immune system.
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PMID:Oral manifestations of primary immune deficiencies in children. 1959 8

Adenosine deaminase deficiency (ADA) is a rare, inherited disorder of purine metabolism characterized by immunodeficiency, failure to thrive and metabolic abnormalities. A lack of the enzyme ADA allows accumulation of toxic metabolites causing defects of both cell mediated and humoral immunity leading to ADA severe combined immune deficiency (SCID), a condition that can be fatal in early infancy if left untreated. Hematopoietic stem cell transplant is curative but is dependent on a good donor match. Other therapeutic options include enzyme replacement therapy (ERT) with pegademase bovine (PEG-ADA) and more recently gene therapy. PEG-ADA has been used in over 150 patients worldwide and has allowed stabilization of patients awaiting more definitive treatment with hematopoietic stem cell transplant. It affords both metabolic detoxification and protective immune function with patients remaining clinically well, but immune reconstitution is often suboptimal and may not be long lived. We discuss the pharmacokinetics, immune reconstitution, effects on systemic disease and side effects of treatment with PEG-ADA. We also review the long-term outcome of patients receiving ERT and discuss the role of PEG-ADA in the management of infants and children with ADA-SCID, alongside other therapeutic options.
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PMID:Pegademase bovine (PEG-ADA) for the treatment of infants and children with severe combined immunodeficiency (SCID). 1970 20

A 31-year-old African American woman with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) (recent CD4 count of 66/mm) presented to the emergency room with a tension pneumothorax that required an emergent chest tube placement. Computed tomography scan showed fungus balls in multiple lung cavities and surrounding infiltrates. The patient showed remarkable improvement with voriconazole suggesting aspergillosis. However, the patient was serologically negative for Aspergillus and other common fungal infections. Because of a persistent air leak, surgical intervention was needed. The histological finding was consistent with invasive mycosis, and cultures were positive for Scedosporium apiospermum. Literature review showed that, among patients with HIV/AIDS, Scedosporium can present from focal localized to systemic disease, is resistant to traditional antifungal agents, and may respond to prompt management with voriconazole.
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PMID:Lung scedosporiosis in human immunodeficiency virus/acquired immunodeficiency syndrome. 2022 Mar 40

Cladophialophora boppii is a black yeast-like fungus that up to now has been only rarely described as a cause of human infection and whose role as a pathogen was not established despite its repeated isolation and genetic identification in these reports. Here we report the first case of a verified toenail infection caused by this fungus in a woman without any systemic disease or evidence of immunodeficiency. Identical dark molds were isolated from the same toenail at three points of time. Species identification was performed by scrutinizing the isolates morphologic, physiologic and genetic characteristics which resulted in their identification as Cladophialophora boppii. Oral treatment with terbinafin plus topical ciclopiroxolamine was effective.
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PMID:Toenail infection by Cladophialophora boppii. 2095 Feb 20


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