ABSTRACT
In twelve rheumatoid arthritis (RA) patients, receiving only nonsteroid anti-inflammatory therapy, superoxide anion (02-) generation by polymorphonuclear leucocytes (PMNs) was assessed by Cytochrome C reduction. The 02- production by non-activated PMNs in RA patients was significantly higher than in healthy controls. The 02- production by PMNs activated by zymosan and phorbol myristate acetate was significantly lower than in controls. PMNs from controls preincubated with rheumatoid sera generated higher 02- levels than the same PMNs incubated in normal sera.
Subject(s)
Arthritis, Rheumatoid/blood , Neutrophils/metabolism , Superoxides/metabolism , Adult , Aged , Arthritis, Rheumatoid/metabolism , Cytochrome c Group/metabolism , Female , Hot Temperature , Humans , Male , Middle Aged , Neutrophils/physiology , Osteoarthritis/blood , Osteoarthritis/metabolism , Oxidation-Reduction , Superoxide Dismutase/pharmacology , Tetradecanoylphorbol Acetate/pharmacology , Zymosan/pharmacologyABSTRACT
EBV infection may have a wide range of clinical consequences: it is often asymptomatic; some cases-generally adolescents-develop the classic "infectious mononucleosis" syndrome; in rare cases the illness takes a severe, fatal course. EBV is also implicated in some geographic areas, in Burkitt' lymphoma and naso-pharyngeal carcinoma. Recent research has enrich our knowledge on the viral genome and the various viral antigen, but some problems are still unsolved. The "normal" immunological response to EBV infection is briefly summarized; then the abnormal immunological pattern is considered in relation to some conditions such as age, congenital and acquired immunodeficiencies and unusual clinical syndromes. Recently a group of not well defined, persistent illnesses (fever, fatigue, headache etc.) has been correlate to a "chronic" EBV infection. It becomes more and more evident that different clinical manifestations of EBV infection are always connected with a particular immunological response; between the "normal" cases and those with well defined immunodeficiency probably large group exists in which minor immunological abnormalities are responsible for a partial derepression of the virus.
Subject(s)
Herpesvirus 4, Human , Virus Diseases/immunology , Aging/immunology , Autoimmune Diseases/immunology , Hepatitis/immunology , Humans , Immunologic Deficiency Syndromes/immunology , Pulmonary Fibrosis/immunologyABSTRACT
There are two distinct forms of multiple carboxylase deficiency. A neonatal onset form is due to deficiency of holocarboxylase-synthetase. A later onset form in which neurological abnormalities are seen as well as those of the skin and hair is due to biotinidase deficiency. It is the purpose of this report to describe a patient with biotinidase deficiency who presents bilateral optic atrophy. The dosage of biotinidase enzyme in the patient's serum and in other members of his family confirms the autosomal recessive transmission of this condition.
Subject(s)
Amidohydrolases/deficiency , Multiple Carboxylase Deficiency/complications , Optic Atrophy/etiology , Alopecia/complications , Biotin/therapeutic use , Biotinidase , Child, Preschool , Genes, Recessive , Humans , Keratoconjunctivitis/complications , Male , Multiple Carboxylase Deficiency/drug therapy , Optic Atrophy/drug therapyABSTRACT
A patient with biotinidase deficiency was studied in whom the first admission to hospital for acidosis occurred at 5 years of age. Sensorineural abnormalities of the optic and auditory nerves antedated diagnosis and treatment with biotin, and these sensory losses did not resolve with treatment. The other clinical manifestations of the disease were highly responsive to biotin. Biotinidase was assayed using 14C-labeled natural substrate. The activity in the patient approximated 1% of the control level.
Subject(s)
Amidohydrolases/deficiency , Multiple Carboxylase Deficiency/enzymology , Amidohydrolases/blood , Biotin/therapeutic use , Biotinidase , Child , Female , Humans , Male , Multiple Carboxylase Deficiency/drug therapyABSTRACT
Two cases are reported of neonatal meningitis caused by Enterobacter cloacae. The two patients are alive; one is perfectly well, the other resulted in hydrocephalus. Both children came from the same neonatal Unit where the were given phototherapy. The importance of nosocomial infections is outlined and different aspects related to the etiology, the environment and the host (particularly immunologic deficits) are briefly reviewed.