Aminoaciduria in handicapped children: a study using ion-exchange chromatography as a screening test.

The aminoacid excretion levels of 59 normal and 75 physically and/or mentally handicapped children were measured with ion-exchange chromatography. A computer-assisted statistical analysis of the results from the normal population was used to calculate an approximating frequency distribution. 18 patients were found to be excreting one or more aminoacids above the normal 99.75th or 100th percentiles. They included 1 with dibasic aminoaciduria; 1 six-year-old with phenylketonuria; 1 with hyperphenylalaninuria; 5 with cystathioninuria; 2 with hyperglycinuria; 1 with hyperglycinuria and hypertaurinuria; 5 with hypertaurinuria; 1 excreting high levels of taurine, serine, tyrosine, and histidine, and 1 with asparaginuria.

Long-term therapy with sodium cromoglycate (intal, lomudal or aarane): effects and side effects.

Review of 72 atopic asthmatic children from the age of three years demonstrated that the long-term use of sodium cromoglycate (Intal, Lomudal or Aarane) produced no serious side effects clinically or on pathological testing. Almost all the children improved, especially in their heights and weights. There is some evidence that girls showed a better response than boys. A possible explanation of growth improvement is discussed and it is concluded that SCG not only prevents asthmatic symptoms but it gives asthmatic children the best chance of maturing normally.

Yeast opsonization defect and immunoglobulin deficiency in severe infantile dermatitis (Leiner's disease).

The defect in Leiner's disease, which presents in early infancy with extensive dermatitis, diarrhoea, and failure to thrive, has been attributed to a defect of the fifth component of complement (C5). We report 2 brothers with extensive dermatitis and dysgammaglobulinaemia. Both died. The older showed symptoms of Leiner's disease: C5 tests were not performed. The younger had extensive dermatitis and was found to have the C5 defect. He developed normally, but died suddenly with pertussis. We postulate that the C5 defect is not the sole cause of Leiner's disease as has been suggested, but that hypogammaglobulinaemia or other lymphoid deficiency is also required for its expression.

Cell-mediated immunity in patients with cystic fibrosis.

Leucocytes from 26 patients with cystic fibrosis (CF) and 18 healthy controls were investigated by migration inhibition induced by a variety of antigens. In patients with CF cell-mediated immunity was found to human lung and pancreatic tissue extracts as well as to Aspergillus fumigatus, Pseudomonas aeruginosa, and food antigens but not to brain, heart, or kidney. Those patients with the severest form of the disease had the greatest impairment of cell-mediated immunity, but this impairment could be reversed by steroid treatment. Cell-mediated cytotoxicity may also be concerned in the pathogenesis of CF.

Immune complexes in cystic fibrosis.

Circulating immune complexes were detected in serum and sputum of patients with cystic fibrosis (C.F.). There were extensive deposits of immunoglobulins and complement immune complexes in several of the C.F. organs, especially the respiratory and gastrointestinal tracts, but not in the kidneys. Significant concentrations of IgG and of complement complexes could be eluted from the lungs of the C.F. patients but not from those of controls. Studies involving immunoabsorption, autoradiography, and molecular sieving through Sephadex G-200 columns identified both bovine serum albumin and staphylococcal alpha-haemolysin as two of the antigens present in the immune complexes. The sedimentation constant of the immune complexes was about 8S to 11S. The clinical significance of these immune complexes and the wide variety of antibodies detected in C.F. patients are discussed.