Zellweger syndrome with severe malnutrition, immunocompromised state and opportunistic infections
نویسندگان
چکیده
منابع مشابه
PAPILLON-LEFE\'VRE SYNDROME: NEUTROPHIL MOTILITY AND KILLING DEFECT IN A CHILD WITH RECURRENT SEVERE INFECTIONS
A case of palmoplantar hyperkeratosis with periodontosis and a history of recurrent severe pyoderma, pneumonia and multiple liver abscesses is described in a 12 year old girl. The patient demonstrated neutrophil dysfunction characterized by decreased random migration and chemotaxis and defective bactericidal activity. The exact immunopathological mechanism for susceptibility to infections i...
متن کاملCommentary: severe varicella/zoster and adenovirus infections in immunocompromised patients.
of symptoms, and the late onset ofthe vesicular rash. As a result of our experience in this case we recommend that varicella zoster antibodies should be measured in all patients receiving methotrexate and, if the result is negative, patients should be advised to seek medical attention when there is contact with chickenpox so that passive immunisation with varicella zos-ter immunoglobulins can b...
متن کاملOpportunistic Infections in Patients with the Acquired Immunodeficiency Syndrome
hemorheology. NMDA receptors, and calcium channel regulation, our ability to treat acute stroke has progressed more slowly than we would otherwise like. William Osler once remarked, "Know syphilis in all its manifestations and relations, and all things clinical will be added unto you." The same advice now holds true for AIDS-it affects every organ system and requires countless forms of therapy ...
متن کاملMR of Zellweger syndrome.
PURPOSE To determine characteristic MR imaging features of Zellweger syndrome. METHODS Clinical records, laboratory records, and MR studies of six patients with Zellweger syndrome were reviewed retrospectively. MR studies were examined for the state of myelination; the presence, extent, and morphologic appearance of cerebral cortical anomalies; the status of the cerebellar cortex, basal nucle...
متن کاملZellweger syndrome (cerebro-hepato-renal syndrome).
A syndrome with distinctive clinical features affecting brain, liver and kidneys was described by Bowen et al in 1964 1 and Smith et al. in 1965 2 . In 1973 Goldfisher et al 3 has reported that peroxisomes were absent in the liver and kidneys of affected children. More recently lack of dihydroxyacetone phosphate acyletransferase (DHAP-AT) a peroxisomal enzyme with a major role in glycerol ether...
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ژورنال
عنوان ژورنال: BMJ Case Reports
سال: 2016
ISSN: 1757-790X
DOI: 10.1136/bcr-2015-214283