[A global intervention program for institutionalized demented patients]. / Méthode de prise en charge globale non médicamenteuse des patients déments institutionnalisés.

INTRODUCTION: Over the last decades many approaches have been developed to manage cognitive and behavioral disturbances in dementia. The present work describes a global intervention program carried out with moderately to severely demented institutionalized patients. The aims of the intervention program are to stimulate and maintain the preserved abilities of demented patients in a supportive context, to decrease the behavioral disturbance and to avoid burnout of care-unit staff. METHODS: This intervention combines different means: psychosocial care (validation therapy, social interaction), cognitive stimulation (memory and verbal training), and motor and sensitive stimulation. The global intervention program requires a special trained team composed of a supervisor, six aid-nurses, an occupational therapist, a speech therapist, a psychomotor therapist and a psychologist. The team cared for the patients five days per week over a three-month period. Assessments were conducted before and after the intervention program to measure the benefit. RESULTS: Positive effects were shown for cognitive abilities, nutritional problems and staff burnout. However, due to the small sample size for this study, more research is needed to verify the effectiveness of this global intervention program, particularly the implications for nutrition. CONCLUSION: This global intervention combined with pharmacological treatment seems to be useful for managing psychological and behavioral disorders of institutionalized demented patients.

Trypsin-binding immunoglobulin G and associated antigen in cystic fibrosis.

Trypsin-binding immunoglobulin G (TBIgG) is found in the sera of a high proportion of patients with cystic fibrosis. We previously reported that TBIgG preferentially binds human cationic trypsin rather than trypsin from other animal species. Binding affinity is enhanced by complex formation with bovine pancreatic trypsin inhibitor, which is known to induce characteristic conformational modifications in the active site region of the trypsin molecule. To identify the human trypsin-like antigen associated with TBIgG, we have studied the effects of conformational changes of cationic trypsin induced by limited proteolysis based on competitive binding studies. It is shown that the most likely TBIgG-related self-antigen is an 11,000-dalton fragment that is a cleavage product of the complex formed by trypsin and alpha 1-protease inhibitor. This result emphasizes the occurrence of circulating trypsinogen activation and is interpreted to be a consequence of the protease-antiprotease imbalance, which has been well documented by previous investigators in cystic fibrosis and also in other lung diseases associated with an inflammatory state.

Excretion of cobalamin and haptocorrin in the meconium of cystic fibrosis, premature, and control neonates.

Excretion of haptocorrin (R binder), cobalamin, and other corrinoids was studied in meconium from cystic fibrosis (n = 4), premature (n = 3), and control neonates (n = 13). Corrinoids content was 1.67 +/- 0.92 pmol/mg protein in meconium of cystic fibrosis (CF) neonates but only 0.33 +/- 0.37 and 0.48 +/- 0.47 pmol/mg protein, respectively, in that of prematures and controls. Considering its molecular mass (110,100 +/- 10,100) and its mean isoelectric point (3.67 +/- 0.20), haptocorrin remained undergraded in the meconium of CF neonates whereas it was partially degraded in the meconium of prematures and in most of the meconium from controls. Sequestration of cobalamin by undergraded haptocorrin can explain its increased excretion in CF meconium. Cobalamin-binding capacity of haptocorrin was 22.13 +/- 15.50 pmol/mg protein in CF meconium and about 400-fold lower in meconium of prematures and controls. This may correspond to a fetal intestinal hypersecretion in cases of CF.

Amniotic immunoreactive trypsin in pregnancies with normal and pathological outcomes.

Immunoreactive trypsin (IRT) has been studied in amniotic fluid as a possible complementary test substance for the prenatal diagnosis of cystic fibrosis (CF). 219 normal amniotic fluids have been tested in order to establish the normal ranges from 14 to 40 gestational weeks (g.w.). The IRT level increases from g.w. 14 to 19, remains stable from g.w. 19 to 25 and then decreases rapidly to low levels. A retrospective study of 4 presumed CF fluids, as determined by abnormal alkaline phosphatase levels, showed decreased IRT values in 3 out of 4 fluids. The difference between the mean value and the normal mean was not significant. The prospective study allowed us to test 2 fluids from pregnancies affected by meconium ileus. IRT level was highly elevated, 530 times the normal mean value at g.w. 34 for a non-CF fetus and 18 times the normal mean value for a CF fetus at g.w. 31. In 4 out of 5 cases of fetal severe intra-uterine growth retardation, IRT levels appeared mildly elevated. Other abnormalities are reviewed.

Effect of pancreatic extracts on the faecal excretion and on the serum concentration of cobalamin and cobalamin analogues in cystic fibrosis.

A malabsorption of crystalline labelled cobalamin is observed in 100% of cystic fibrosis patients. Using radioisotope dilution assays and molecular sieve gel chromatography, we determined the serum concentration and the faecal excretion of cobalamin and cobalamin analogues in nine cystic fibrosis children before and after 4 days' interruption of pancreatic extract treatment. On chromatography, the unsaturated cobalamin binders of the faecal extracts eluted in two positions with molecular masses of 44 300 and 20 300, corresponding mostly to partially degraded R binders. The amounts of the less degraded form of R binder (molecular mass 44 300) increased significantly after interruption of the treatment. The cobalamin concentration in the serum remained normal after interruption of the treatment but the analogue concentrations in the serum decreased and faecal excretion of cobalamin and analogues increased significantly. These results allowed us to suggest that (1) pancreatic insufficiency in cystic fibrosis is responsible for a decrease in the absorption of digestive analogues induced by a defective degradation of R binders, and (2) cobalamin analogues have a short half-life in blood.