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Biomedical subjects

V Lucidi

Publications and source records attributed to V Lucidi.

At least 37 records · Page 2Linked to original sources
PubMed1993

Down's syndrome and celiac disease: the prevalence of high IgA-antigliadin antibodies and HLA-DR and DQ antigens in trisomy 21.

Patients with Down's syndrome (DS) or celiac disease (CD) have altered immune systems. Autoimmune diseases have been described in both conditions; the coexistence of DS and CD has been occasionally reported, but a clear relationship has not been definitely established. In this study we determined IgA antigliadin antibodies (IgA-AGA) in 155 children with DS, and the results were compared with those of the control groups formed by 320 children affected by upper-respiratory tract infections and 115 children with gastrointestinal symptoms but with normal jejunal mucosa. High IgA-AGA levels were found in 26% of DS patients, in 1% of the first control group and in 10% of the second control group. Such differences are statistically significant. Twenty-one DS patients with high IgA-AGA levels and gastrointestinal symptoms underwent jejunal biopsy, and total villous atrophy was found in seven of them (33.33%). HLA-DR and -DQ antigens were also determined in 75 DS patients (20 with high and 55 with normal IgA-AGA levels), and the percentages of the different phenotypes were compared in the two groups and with those of a control group. No statistically significant difference was found, but DR3, DR7, and DQ2 alleles were always present in DS patients with jejunal atrophy. Our study confirms the data reported in the literature about higher levels of IgA-AGA in DS patients and the relatively high incidence of CD in this group of patients.

Adolescent↗
PubMed1990

[Changes in the prostanoid content and the population of endocrine cells in jejunal biopsies from celiac children].

Prostanoid content prostaglandin E2 (PGE2) and thromboxane B2 (TxB2) and endocrine cells population were evaluated in jejunal biopsies from celiac children; findings were compared to active celiac patients on a challenge diet. Patients were divided as follows: Group A: 14 children with active untreated celiac disease; Group B: 7 celiac children on gluten challenge who had received diet therapy for the past 2 years; Group C: 8 normal control children. Jejunal biopsies were used for endocrine cell population measurement by immunocytochemistry, using a specific marker (chromogranin), and for prostanoid radioimmunological evaluation. The quantitative assessment of the endocrine cell population in Groups A and B revealed a significantly higher number of endocrine cells (20 +/- 11.5; 18.4 +/- 9.8 n. cell/visual field respectively) compared to Group C (8.44 +/- 2.3 n. cell/visual field) (p less than 0.05). In the jejunal extract the PGE2 content (341.8 +/- 82.3) ng/g) for Group A biopsies was significantly higher than that of Group C biopsies (93 +/- 23 ng/g) (p less than 0.05. The PGE2 content (69.4 +/- +13.2 ng/g) for Group B did not show any statistically significant change. In contrast, TxB2 content in jejunal biopsies from all three groups was not significantly different.

APUD Cells↗
PubMed1990

[Intestinal absorption of vitamin E in children with atrophy of the jejunal mucosa].

The intestinal absorption of vitamin E is often thought to be closely correlated to that of fatty acids, but the biochemical mechanisms involved in the process are still not clear. For this reason, the present study aimed to assess the absorption of triglycerides and vitamin E, following oral administration, in 11 children with suspected malabsorption syndromes. Intestinal biopsy in 9 children showed the presence of a flat mucosa, whereas no changes were present in 2 patients. The results confirm the inverse correlation between the absorption of triglycerides and vitamin E, as if tocopherols used an independent system of transport. These findings contrast with those reported in the literature perhaps due to the low level of precision of colorimetric techniques previously used to assay vitamin E.

Atrophy↗
PubMed1987

Prostanoids in jejunal biopsy specimens of celiac children with active disease and on challenge diet. Radioimmunologic evaluation.

Prostanoid generation (prostaglandin E2 and thromboxane B2) in jejunal biopsy specimens from celiac patients was evaluated, comparing celiac patients with celiac patients on challenge diet and controls. Generation of prostaglandin E2 in jejunal specimens from 14 children with active celiac disease was significantly higher (341.8 +/- 82.3 ng/g; mean +/- SEM) than that from 7 celiac patients on gluten challenge diet (69.4 +/- 13.2 ng/g) or 8 normal children (92 +/- 23 ng/g) (p less than 0.05). In contrast, thromboxane B2 generation in jejunal specimens from all three groups did not show any statistically significant variation. Our results indicate that prostaglandin E2 generation is not merely related to the activity of clinical symptoms, since patients receiving gluten challenge had prostaglandin E2 levels that did not differ from those of controls.

Adolescent↗
PubMedDate not supplied

Pancreatic cystosis in cystic fibrosis: case report.

We describe a 19-year-old cystic fibrosis patient, with pancreatic insufficiency since the age of 4 who presented at the age of 13 with postprandial abdominal pain. Ultrasonography and computed tomography showed several pancreatic cysts that progressively increased in diameter over 6 years. The lack of association with clinical and biochemical signs of acute pancreatitis is highlighted.

Adult↗
PubMedDate not supplied

Benign intracranial hypertension in an older child with cystic fibrosis.

Despite having normal height and weight, a 6-year-old girl had frequent bowel movements and slight recurrent chest infections since the age of 4 years and headache for 1 year. The patient appeared healthy, but examination of the ocular fundus revealed papilledema. Cranial computed tomography appeared normal. Lumbar puncture disclosed an elevated opening cerebrospinal fluid pressure, with normal biochemical, cellular, and bacteriologic findings. Laboratory investigations indicated pathologic steatorrhea, elevated electrolytes in 3 sweat tests, and low serum levels of vitamins A and E. The diagnosis of pseudotumor cerebri in a patient with cystic fibrosis was made. After treatment with prednisone (1 mg/kg/day), pancreatic extracts, and vitamin supplements, headache and papilledema resolved and serum vitamin A and E levels subsequently became normal. Older children with cystic fibrosis rarely have benign intracranial hypertension, but when present it is often due to hypervitaminosis during correction of malnutrition. In this child, pseudotumor cerebri and associated hypovitaminosis improved after combined corticosteroid and vitamin treatment.

Child↗
PubMedDate not supplied

Comparison of two enteric coated microsphere preparations in the treatment of pancreatic exocrine insufficiency caused by cystic fibrosis.

BACKGROUND: Pancreatic exocrine insufficiency is a common condition in patients with cystic fibrosis. Large amounts of pancreatic enzyme supplements are required to reduce malabsorption but patient compliance is not always optimal. AIMS: To compare patients' preference and the efficacy of two enteric coated microsphere preparations in patients with cystic fibrosis. PATIENTS: Patients with pancreatic exocrine insufficiency due to cystic fibrosis. METHODS: Patients were assigned to the crossover treatment with Creon or Pancrease for 1 week and then to the alternative treatment. Patients had to follow a fixed diet (at least 2 g fat/kg) and had to assume 1000 units lipase/g fat. The evaluation parameters were: patients' preference, acceptance of therapy, stool fat excretion, stool weight, gastrointestinal symptoms, and tolerance. RESULTS AND CONCLUSIONS: Of the 33/60 patients who expressed a preference for one of the two treatments, 30 preferred Creon while only 3 patients preferred Pancrease (p<0.001). No difference between the two treatments was observed regarding stool characteristics, gastrointestinal symptoms and tolerance. The mean number of capsules taken daily was reduced by 35% with Creon. The results of this study showed a preference in favour of Creon probably due to the reduction of daily capsule intake of 35%, supporting digestion as well as Pancrease.

Adolescent↗
PubMedDate not supplied

Phenotype/genotype correlation and cystic fibrosis related diabetes mellitus (Italian Multicenter Study).

BACKGROUND: A genotype/phenotype correlation between early onset cystic fibrosis related diabetes (CFRD) and the N1303K mutation of the CF gene was previously identified in a small series of 28 CFRD patients, out of 313 CF patients. PATIENTS AND METHODS: In order to confirm the observation, data of 141 CFRD patients out of 1,229 CF patients attending 14 Italian CF centers were collected. All patients were older than 10 years and had been genotyped. RESULTS: DeltaF508 was the most frequent mutation (147/282 alleles: 52%) and N1303K the second most frequent mutation (18/282 alleles: 6.3%) in CFRD patients, without significant difference as compared with CF patients without DM (52% vs 48.6% and 6.3% vs 5.1%, respectively). W1282X was the third most frequent mutation in CFRD patients, more frequent than in CF patients without DM (5.3% vs 2%; p<0.001). CONCLUSIONS: Unlike the previous study, we did not find a higher frequency of the N1303K mutation in CFRD patients; moreover, data from this large CF series showed a significant correlation between the W1282X mutation and CFRD.

Adolescent↗
PubMedDate not supplied

[Italian Cystic Fibrosis Registry: 10 years of activity].

Cystic Fibrosis (CF) is a recessive autosomic genetic disease with an incidence in mediterranean countries of about 1:3500 born alive. In Italy the considerable genetic variability makes it difficult to identify all the homozygous subjects and, consequently, to estimate the incidence of the disease in healthy carriers. The disease is evolutive and affects various systems, most of all the respiratory and gastrointestinal systems. Not many years ago, when the clinical definition of CF was first introduced, average survival did not exceed the pediatric age. Nowadays with ever advancing diagnostic and therapeutical techniques many CF patients survive until an adult age. It is therefore necessary to plan adequate health service interventions so as to satisfy as much as possible the needs of both the patients and their families. To this end data collected since 1.1.1988 by the Italian registry for CF (year of birth, sex, region of birth and residence, diagnosis procedures, results of sweat test, pancreatic insufficiency, DNA analysis, status: alive, dead, lost to follow up) of all the patients, diagnosed in the 18 Reference Centres and the 3 local Centres for CF, have proved to be extremely useful. Since the birth of the Registry on 31.12.1997, data relating to 2458 patients alive on 1.1.1988 and 1159 born during the last ten years, for a total of 3617 subjects (1756 females and 1861 males), have been recorded. As already mentioned a considerable increase in life expectancy of CF patients (from 1988 to 1990 the average age of death was 14 years, from 1994 to 1997 it was 19) and a consequent increase in the percentage of adult patients have been observed.

Adolescent↗
PubMedDate not supplied

[The correlation between the genotype and the clinical expression of cystic fibrosis].

The authors report a phenotype-genotype correlation in a population of patients from center and south Italy affected by cystic fibrosis (CF). Thirteen (21%) of patients with pancreatic insufficiency (PI) were homozygous for delta F508, seventeen (27%) were delta F508 heterozygous with an associated unknown allele. Two other described mutations (N1303K, G542X) were also found in a heterozygote status and associated with a severe clinical expression of disease.

Adolescent↗
PubMedDate not supplied

[IgA-class antigliadin antibodies in the screening and follow-up of celiac disease patients].

IgA antigliadin antibodies (IgA-AGA) have been determined with an enzyme immunoassay in 2.141 pediatric patients. High levels of IgA were found in 98% of 53 celiac patients (1st biopsy), in 81% of 16 celiac patients after gluten challenge, while high levels of these antibodies were not found in 200 patients on gluten-free diet. Moreover high levels of IgA-AGA were found in 29% of 48 patients with normal jejunal biopsy and in 4% of 1.824 patients with gastrointestinal problems other than celiac disease. Our results confirm the data report in literature about the sensibility and the specificity of the IgA-AGA dosage as a screening test for celiac disease, but the possibility of false pathological and false normal values confirms the intestinal biopsy, as the main procedure for the diagnosis of celiac disease.

Antibodies↗
PubMedDate not supplied

[Ascites as an unusual manifestation of chronic granulomatous disease in childhood].

Ascites is a late and uncommon symptom of Chronic Granulomatous Disease (CGD) of childhood following liver damage with portal hypertension due to hepatic granulomatosis. The Authors describe two patients with CGD, in whom ascites was the main symptom in the first case and the initial symptom in the second case. Ascites in these cases was not due to liver fibrosis but it was a consequence of primary bacterial peritonitis.

Ascites↗
PubMedDate not supplied

[Immunofluorescence in the determination of gliadin antibodies in celiac disease].

IgG gliadin antibodies (AGA-IgG) were detected by immunofluorescence in 78 celiac children diagnosed by jejunal biopsy. AGA-IgG were also detected in patients affected by other gastrointestinal disease, in patients with diabetes, in health children and in children with normal mucosa. AGA-IgG were found in 81% of celiac patients, while they were not detected in patients affected by other intestinal or extraintestinal diseases, neither in healthy controls. It is conclude that determination of AGA-IgG is a very specific and sensitive enough screening test before small intestinal biopsy.

Adolescent↗