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.
An Italian multicenter study has been carried out in order to obtain data on survival and prognostic factors in CF. We report data from four CF Centers: Milan, Neaples, Cagliari, Rome, Anagraphic ...data, mode of presentation and of diagnosis were collected, using CFDB database, from 536 CF pts. 39 pts had meconium ileus (7.3%); 269 (50%) were diagnosed before 12 months of age. 27 pts were lost to follow-up (5%); and 65 pts died (12%). Statistical analysis was performed by Kaplan-Meier survival curves compared by Logrank test.
A stimulated altitude of 7000 m was applied in rats chronically exposed to hypoxia in a hypobaric chamber. In these experimental conditions the contralateral ventricle not exposed to pressure ...overload (that is, the left) showed a significant degree of hypertrophy. The hypoxic stimulus did not affect systemic artery pressure or the heart rate. Chronic adrenergic stimulation performed in the rat treated with low doses of isoprenaline (a direct stimulating adrenergic drug) or with tyramine (primarily a releaser of endogenous catecholamines) induced hypertrophy of the right ventricle, left ventricle, and interventricular septum without modifying the haemodynamic parameters mentioned above. The development of ventricular hypertrophy in the absence of pressure overload is stressed. A role for neurohumoral factors (that is, adrenergic stimulation and catecholamines) in the development of ventricular hypertrophy is suggested.
The aim of the present study was to verify the validity of diagnostic criteria developed by the NDDG for the diagnosis of diabetes mellitus in old age. One hundred and fifty-one ambulatory old ...(range: 66-77 years) subjects (group A) underwent OGTT showing the following results: 33% normal, 12% non-diagnostic, 23% impaired glucose tolerance (IGT), 32% diabetic-type tolerance (DT). In addition, 84 subjects (group B) selected from 1978 to 1982 (42 aged 51-60 years, 30 aged 61-70, and 12 aged 71-80) with abnormalities of glucose tolerance during OGTT (IGT or DT) were asked to control their fasting plasma glucose every month during 1984. In group B a significant correlation between DT and subsequent development of fasting hyperglycemia was observed only in the subjects of the 6th and 7th decades of age. On the contrary, no subjects aged 71-80 years developed fasting hyperglycemia. The authors suggest that a high prevalence of abnormalities in glucose tolerance according to NDDG exists in old age which cannot be considered evidence of a true diabetes mellitus being unpredictive of a progression towards fasting hyperglycemia.
Ten non-obese type 2 diabetic patients with secondary failure to sulfonylureas received an intensive insulin therapy (four doses schedule) for 90 days. The glycaemic control was poor at entry (HbA1c ...= 11.7 +/- 1.2%) and ameliorated significantly after insulin (HbA1c = 7.1 +/- 0.7%, p less than 0.01). The reintroduction of the sulfonylurea after insulin withdrawal resulted in a persistent satisfactory long-term control (300 days) in all, but two diabetics responded no more after about 3 and 4 months of clinical remission (good control on sulfonylurea). Both basal and stimulated (iv glucagon and mixed meal) beta-cell secretory activity increased significantly at 3 months and declined thereafter without falling below baseline values. Three months of strict metabolic control seem to restore the sensitivity to sulfonylurea by enhancing beta-cell secretory activity in non-obese type 2 diabetic patients.
This study was undertaken to assess the mechanism by which prostaglandins of the E series inhibit glucose-induced insulin secretion in man. Acute insulin response (mean change 3-10 min) to iv glucose ...(0.33 g/kg) was decreased by 40% during the infusion of prostaglandin E2 (10 micrograms/min) and glucose disappearance rates were reduced (P less than 0.05). Insulin response to arginine (5 g iv) and tolbutamide (1 g iv) were not affected by the same rate of prostaglandin E2 infusion. The inhibitory effect of prostaglandin E2 on glucose-induced insulin secretion was prevented by theophylline (100 mg as a loading dose followed by a 5 mg/min infusion), a drug that increases the intracellular cAMP concentrations by inhibiting phosphodiesterase activity. Our data suggest the involvement of the adenylate cyclase system in the inhibitory action of prostaglandin E2 on glucose-induced insulin secretion in man.
Extract: A retrospective approach has been adopted to investigate the frequency and duration of breastfeeding and the time of gluten introduction in the diet in 216 celiac children and their healthy ...siblings of 3 different Italian centers (Naples, Milan, and Turin). In this matched case-control study, the selected controls were healthy siblings of the cases. Children formula-fed from birth or breast-fed for less than 30 days were found to have a relative risk of developing symptoms of celiac disease 4 times higher than children breast-fed for more than 30 days. To further investigate the effect of the duration of breastfeeding as a possible protecting factor, the linear trend for different periods of breastfeeding was tested and found to be highly significant. Therefore, increased duration of breastfeeding is associated with decreased risk of developing celiac symptoms. There was no apparent relationship between early introduction of gluten into the diet and frequency of celiac disease. These findings are consistent with the hypothesis that a wider diffusion of breastfeeding is a factor underlying the recently reported decrease of the incidence of celiac disease in children. (author/wz)
