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Biomedical subjects

B Santini

Publications and source records attributed to B Santini.

At least 19 recordsLinked to original sources
PubMed2001

Diagnostic accuracy of fecal elastase 1 assay in patients with pancreatic maldigestion or intestinal malabsorption: a collaborative study of the Italian Society of Pediatric Gastroenterology and Hepatology.

Several reports have indicated that fecal elastase-1 (EL-1) determination is a new, sensitive, and specific noninvasive pancreatic function test; however, very few patients with malabsorption due to small intestine diseases have been included in the previous studies. The aim of the study was to compare the diagnostic accuracy of fecal EL-1 and fecal chymotrypsin (FCT) in distinguishing between pancreatic maldigestion and intestinal malabsorption. Three groups of subjects were studied: group A included 49 patients with known cystic fibrosis (25 males, median age 5 years); group B included 43 subjects with various small intestine diseases (17 males, median age 6 years); and group C included 45 children without any history of gastrointestinal disease (22 males, median age 5 years). In all patients, stools were collected for 72 h on a standard diet and fecal EL-1, FCT, and steatocrit tests were performed. Both EL-1 and FCT were below normal limits in all CF patients with pancreatic maldigestion not treated with pancreatic enzyme (100% sensitivity for both assays); El-1, but not FCT, was also below normal in all the CF patients with pancreatic maldigestion treated with pancreatic extracts. Both EL-1 and FCT values in the CF group were significantly lower than in subjects with various small intestinal diseases and in children without any history of gastrointestinal disease (P < 0.0001). FCT, but not EL-1, values showed an inverse statistically significant correlation with steatocrit values in the whole CF group (P < 0.001); FCT was below normal in three of four CF patients with steatorrhea on pancreatic enzyme therapy. Both EL-1 and FCT had 100% specificity when calculated in children without any history of gastrointestinal disease; in contrast, specificity was 86% for EL-1 and 76% for FCT if we considered the control group with small intestinal diseases: low EL-1 was observed in two cases of intestinal giardiasis, two cases of short bowel syndrome, one case of celiac disease, and one case of intestinal pseudobstruction; FCT was abnormal in four cases of intestinal giardiasis, three cases of celiac disease, one case of short bowel syndrome, one case of Crohn's disease, and one case of intestinal pseudobstruction. Diagnostic accuracy was 92% for fecal EL-1 and 82% for FCT. Steatocrit values were over the normal limit in 11 patients with small intestine diseases; in 7/11 of these patients at least one of the pancreatic test results was below the normal limit. In conclusions, in patients with CF, fecal EL-1 determination is not more sensitive than FCT in identifying pancreatic maldigestion; however, fecal EL-1 assay is more specific than FCT determination in distinguishing pancreatic maldigestion from intestinal malabsorption.

Adolescent↗
PubMed1997

[Adolescence in patients with cystic fibrosis studied by Rorschach test].

BACKGROUND: The majority of patients with cystic fibrosis today reach adulthood. During adolescence, however, many ask themselves questions regarding their possible future realisation. AIMS: The aim of this study, which was performed using the Rorschach test, was to identify areas of particular problems in a group of CF adolescents monitored by the Pneumology Division of Regina Margherita Children's Hospital in Turin: 26 subjects aged between 14 and 18, including 11 females and 15 males. METHODS: The analysis of the test was based on the use of thought, interior resources, instincts and interpersonal relations. The type of intimate resonance was also taken into account and, lastly, a study of the contents was performed using a self-representation grid. RESULTS: The results obtained show a dishomogeneous profile of the development and quality of intellectual processes faced with the intensity of anxiety, mainly loss anxiety, linked to the disease, even if unrelated to its severity. The integration of emotive and instinctual aspects is difficult: mental development towards adult life appears to be impeded by the predominance of regressive modalities. CONCLUSIONS: On the basis of the study performed, it is possible to hypothesise the need for psychological help aimed at achieving a more evolved psychic organisation, possible as part of an overall management programme by the medical and nursing team.

Adolescent↗
PubMed1997

[Personality structure in children with cystic fibrosis studied using Rorschach test].

BACKGROUND AND AIMS: The recent positive evolution in the development of cystic fibrosis, resulting in the current prospect of survival, has led to the formation of a new population of chronic patients. The characteristics of the disease and treatment (hereditary, early onset, affecting the alimentary tract and airways) may interfere with personality structuring processes. The aim of the study was to identify the psychopathological risk. METHODS: The study was performed using the Rorschach test in a population of children with cystic fibrosis monitored by the Pneumology Division of the Ospedale Infantile Regina Margherita in Turin: 20 children aged between 7 and 13 years old were included in the study, most of whom had been diagnosed before the age of 2. The analysis of tests was focused on the use of thought, interior resources, instincts and interpersonal relations. A study of the contents was also made using a self-representational grid. RESULTS: The results obtained highlight an inhibitory attitude which cannot be attributed to intellectual insufficiency, but rather to the failed attempt to invest in the intellectual sphere, typical of this age range, owing to the intensity of anguish. Children with cystic fibrosis appear to be at an earlier stage of development, marked by emotive coarctation and the use of adult-type defensive methods, tending towards rationalisation. CONCLUSIONS: Oh the basis of this study, it is possible to detect a danger of disharmonious organisation in the personality structuring process of these children. The hospital must therefore offer adequate psychological support for their psychic evolution.

Adolescent↗
PubMed1996

[Home enteral nutrition in pediatric age. Based on the Torino experience].

Enteral nutrition is increasingly used in the management of sick children. Malnutrition may frequently complicate chronic illness in children and nutritional support may be a primary treatment. Particularly children with malnutrition due to certain chronic disorders may need long term enteral nutrition not only in hospital but also at home. We report our experience on home enteral nutrition of 14 patients, aged from 3 months to 15 years. Eleven had a serious weight deficit (< 3 degrees percentile), but none had severe alteration of the biohumoral nutritional parameters. The indication for EN were: in 7 patients cerebral palsy, in 2 cystic fibrosis, and in the other 5 patients congenital heart disease, multiple food intolerance, oesophageal athresia, Werding-Hoffman disease, Costello's Syndrome. Nutritional support was given by nasogastric tube in 6 patients, through gastrostomy in 3 patients and by mouth in 5 patients. We did not see any severe complication due to home enteral nutrition. The practicalities of this nutritional approach are discussed. Although home enteral nutrition was evaluated in a low number of patients, it may have a role in promoting growth of children with inadequate dietary intake or malnutrition and in improving their quality of life.

Adolescent↗
PubMed1993

[Pancreatic extract therapy in exocrine pancreatic insufficiency].

The authors consider the different strategies in long term enzyme replacement therapy in relation to the complex mechanics at the basis of pancreas exocrine insufficiency. This condition occurs in chronic pancreatitis and is present in Cystic Fibrosis, the most common potentially lethal inherited disorder of Caucasians. Pancreatic exocrine insufficiency occurs in the vast majority of cystic fibrosis affected children and is now becoming a frequent pathology in adults for the better life expectancy and the recent acknowledgements in this disease. The principal aims of research in enzyme replacement therapy have been directed at the formulation of products with high enzyme concentration, to the prevention of gastric acid inactivation of enzymes and to the better mixing of the preparations with meals. The authors consider all the different enzyme preparations from pancreatin powder to the 1st. generation of enteric coated tablets and examine the advantages of administering H-2 receptor antagonists or antacids and the possibility of stimulating bicarbonate secretion as an adjunct to pancreatic enzyme replacement therapy. Significant benefits in pancreatic insufficiency therapy have derived from the introduction of enteric coated microspheres which ensure a consistent level of enzymes to reach the duodenum mixed with the meal and which are resistant to gastric acid inactivation as well.

Exocrine Pancreatic Insufficiency↗
PubMed1990

delta F508 deletion in cystic fibrosis in Italian families.

In 20 Italian families with cystic fibrosis (CF), restriction fragment length polymorphisms were detected by five linked markers; a strong linkage disequilibrium is observed between the haplotype B (alleles 2/1 with respect to KM19/XV2c) and CF. The frequency of the delta F508 deletion in CF chromosomes of this sample is 50%. A significant correlation is found between the absence of the delta F508 mutation and pancreatic sufficiency.

Cystic Fibrosis↗
PubMed1988

Efficacy of pancreatic enzyme supplementation in children with cystic fibrosis: comparison of two preparations by random crossover study and a retrospective study of the same patients at two different ages.

Two studies were performed in children with cystic fibrosis (CF) to compare the efficacy and tolerability of pancreatic enzymes prepared as enteric-coated microspheres with that of a conventional enzyme preparation. The parameters evaluated included the following: fecal fat excretion, coefficient of fat absorption, daily caloric intake, percent of diet as fats, proteins and carbohydrates, increase in height and weight, frequency and consistency of stools, palatability of the preparation, and patient compliance. The first study was an open randomized crossover trial of an enteric-coated microsphere preparation (Pancrease) versus conventional pancreatin given alone or with cimetidine. With Pancrease compared to conventional pancreatin, a significant improvement was observed in all the digestive parameters in addition to better patient compliance. In the second study, the response of a group of CF patients given Pancrease for at least 3 months (3-67 months) was retrospectively compared with the response to conventional pancreatin, which had been given to the same patients from 10 months to 8 years earlier. In comparison to conventional pancreatin, Pancrease provided better digestive efficacy and greater increases in the growth rate of teenage patients. With Pancrease, the number of daily dosage units is decreased even when fat intake is increased. No adverse reactions were seen with either of the enzyme preparations used in these studies.

Adolescent↗
PubMed1984

Serum group I pepsinogens level in cystic fibrosis.

Fasting serum pepsinogen 1 level was determined by radioimmunoassay in 30 patients with cystic fibrosis and in on equal number of age-matched healthy children. Serum pepsinogen 1 basal levels were significantly higher (49 +/- 18 ng/ml; p less than 0.01) in cystic fibrosis patients than in controls (38 +/- 12 ng/ml). The increased pepsinogen 1 level did not correlate with the levels of Po2 and Pco2 tensions, fat balance or basal and maximum acid outputs. It is concluded that the mechanisms which lead to a rise of serum pepsinogen 1 in cystic fibrosis are unknown. However these findings could provide useful information with regard to the biology of a disease whose physiopathology is still unknown.

Adolescent↗
PubMed1983

Serum trypsinlike immunoreactivity in cystic fibrosis. An aid in assessing progressive involvement of the pancreas.

Serum trypsinlike immunoreactivity (TLI) was measured in 42 children with cystic fibrosis (CF) and related to age and steatorrhea. The mean TLI value in 106 age- and sex-matched control subjects was 22 +/- 7.2 ng/mL. In patients with CF, TLI showed a widespread distribution (range, 0.3 to 214 ng/mL), and an exponential inverse correlation between TLI and age was found. The same trend in the decline in TLI values with increasing age was evident in patients who were followed up. Reduced TLI concentrations were associated with fat malabsorption, but in younger patients with CF, normal or elevated TLI values did not exclude steatorrhea. Elevated serum TLI levels in patients with CF at an early age might reflect pancreatic ductal obstruction. Tendency for TLI to decrease with advancing age and in patients who are followed up can reflect ongoing degenerative destruction within the pancreatic gland. Serum TLI estimation in patients with CF should be used to monitor the progression of pancreatic impairment.

Adolescent↗
PubMed1983

HLA-DR3 and DR7 in coeliac disease: immunogenetic and clinical aspects.

The association of HLA-A,B,C, DR polymorphisms and of Bf and GLO with coeliac disease was analysed in 100 Italian children. Primary involvement of HLA-DR3 and DR7 is apparent, while specificities of nearby loci are probably associated secondarily, because of linkage disequilibrium. Direct assessment of D/DR genotype through family studies and mixed lymphocyte cultures led to the recognition of two high risk genotypes DR3/3 and DR3/7, and of two lower risk genotypes DR3/X and DR7/X. The different weight of the HLA-dependent genetic factors is to some extent correlated with the clinical and immunological parameters, suggesting that the low-risk genotypes induce a milder expression of coeliac disease. Furthermore, other genetic factors, such as sex, appear to contribute to the penetrance of the disease, especially in the case of DR3/X and DR7/X.

Adolescent↗