A. G. Ugazio

Immunodeficiency and syndromes: a nosographic approach.

Immunodeficiency disorders can be classified on clinical grounds into two broad groups according to whether all features are the result of the immune defect (immunodeficiency syndromes) or whether many, even prominent, features cannot be explained by the immune defect (syndromes with immunodeficiency). X-linked agammaglobulinemia and X-linked chronic granulomatous disease are paradigmatic examples of immunodeficiency syndromes.Despite some overlap (for instance extra-immune symptoms, although minor, are present in several variants of severe combined immunodeficiency and chronic granulomatous disease) immunodeficiency syndromes and syndromes with immunodeficiency are easily distinguishable.Together with the pathogenetic classification of the WHO, the present approach to a clinical classification amplifies the operational concept of immunodeficiency also from a therapeutic point of view.

A new immunoperoxidase assay for Lolium perenne-specific IgE in serum based on the biotin/avidin system (BAS).

A new solid‐phase immunoassay based on the biotin/avidin system (BAS) for measuring scrum Lolium perenne (LP)‐specific IgE antibody is described. LP‐specific IgE was assayed by the BAS assay and RAST for comparison in the sera of thirty‐two normal asymptomatic subjects R AST‐negative for LP and of twenty‐six subjects with hay fever and R AST‐positive for LP. The specificity of the BAS assay for LP‐specific IgE was demonstrated by absorption experiments. An overall agreement of 91% (53/58) was observed between the BAS and RAST and a high correlation (r= 0.87. P < 0.001) was found between the LP‐specific IgE determined by the two methods. The advantages of ihe BAS assay as compared to both the RAST and classical ELISA arc discussed.

Pulmonary hypertrophic osteoarthropathy in a child with late-onset agammaglobulinemia

A case of hypertrophic osteoarthropathy is described in an 8-year-old child with late-onset agammaglobulinemia, bronchiectasis and clubbing of the fingers and toes. The child presented with pain of recent onset in the legs, ankles, and knees and a diagnosis of hypertrophic osteoarthropathy was made on the basis of the radiologic findings. Therefore the differential diagnosis of bone and joint pain in agammaglobulinemic subjects must take into account hypertrophic osteoarthropathy.

Polymorphonuclear function and respiratory syncytial virus infections in children

An impairment of polymorphonuclear functions has been observed during viral respiratory infections (1-3); however, the degree, duration and clinical relevance of the impairment are still controversial. We have studied a panel of polymorphonuclear (PMN) functions in 18 infants with lower respirator), tract infections of known viral etiology in order to investigate the presence of PMN impairment and its possible relationship to the viral etiology or the severity of the disease. The subjects included in the study were 18 infants aged one to 18 months who were hospitalized during the winter of 1981 with lower respiratory tract infections of viral etiology. They were divided into two groups according to the virus isolated: Respiratory Syncytial virus (RSV) was isolated from 11 patients (Group A) and in the remaining seven patients (Group B), the viruses isolated were Adenovirus in two cases, Influenza A virus in three, Parainfluenza III virus in one and Influenza B virus in one. The severity of the disease was assessed by the score proposed by Bruhn and Yeager (4). PMN functions were evaluated in all patients on admission, as well as ten and 30 days later. Thirty-five age-matched children who were hospitalized during the same period for non-infectious diseases served as controls. Chemotaxis, respiratory burst, frequency of phagocytosis, nitroblue tetrazolium (NBT) reduction and Staphylococcus aureus killing were performed as previously described (5). Statistical analysis was performed by the Students t-test. The results are summarized in Table t . Group A (RSV-infected) children showed a severe impairment of all of the PMN functions tested with the exception of NBT reduction; the defects present early in the disease had almost always returned to normal by Day 10. In Group B (other viruses), however, the only abnormality was the ability to kill S. aureus observed at the beginning of the disease. No correlation was found between the degree of PMN impairment and the severity of the d i sease . In conclusion, we observed a severe impairment of many PMN functions during respiratory tract infections due to RSV; this defect was not related to the age or sex of the patients. Other workers have shown an inhibitory effect of RSV on chemotaxis, killing ability and NBT reduction (1); our results failed to show a W inhibition of NBT reduction and confirmed the impairment of chemotaxis and killing ability. Moreover , our results demonstrated that other PMN functions such as respiratory burst and phagocytosis were

Staphylocidal defect of polymorphonuclear leukocytes in a patient with Leprechaunism

A patient with the features of Leprechaunism had a severe and constant defect of bactericidal activity of his phagocytes. We suggest this abnormality might be the basis of the well known recurrence of infections in this syndrome.