An epidemiologic survey of celiac disease in the Terni area (Umbria, Italy) in 2002-2010.

The present work is an epidemiology survey of celiac disease in the province of Terni (Umbria, Italy) in 2002-2010. Data were collected from the Local Health Unit (LHU) 4 (ASL 4), Terni database and were extrapolated from the overall population of 232,540 (as of 2010) by identifying residents with prescription charge exemptions for celiac disease-oriented drugs. Prevalence and incidence analysis over the timeframe being examined showed that prevalence (330 cases in 2010) has consistently been increasing from 2002 to 2010, whereas incidence has remained essentially the same with minor, yearly fluctuations. Both prevalence and incidence were higher in females than in males. Most patients were diagnosed as young adults, with the highest rates in the 10-14, 35-40 and 55-60 age groups. Thus, in the area of investigation, there is evidence for consistent delayed diagnosis, raising the possibility that the atypical form the disease, more difficult to recognize and more likely to escape early diagnosis, may have become increasingly commoner overtime. Because the current prevalence of the disease in the Terni area is estimated to approximate 1%, the anticipated number of cases should amount to 2325, which value contrasts with the currently reported 330 diagnoses. It is suggested that the current illness-defining criteria should be revised so to implement early diagnosis and improve the patients' quality of life and access to treatment.

Study of ethane level in exhaled breath in patients with age-related macular degeneration: preliminary study.

PURPOSE: A variety of factors have been implicated in the pathogenesis of age-related macular degeneration (ARMD), and oxidative stress plays an important role in the onset and progression of the disease. Breath ethane is now considered a specific and non-invasive test for determining and monitoring the trend of lipid peroxidation and free radical-induced damage in vivo. This test provides an index of the patients' overall oxidative stress level. We evaluated the breath ethane concentration in exhaled air in patients with advanced ARMD. METHODS: In this study, we enrolled 13 patients with advanced ARMD and a control group, and a breath analysis was carried out by gas chromatography. RESULTS: The mean ethane level in the ARMD patients was 0.82 ± 0.93 nmol/l (range: 0.01-2.7 nmol/l) and the mean ethane value in the control group was 0.12 ± 0.02 nmol/l (range: 0.08-0.16 nmol/l). The difference between the values of the 2 groups was statistically significant (p < 0.005). Receiver operating characteristic analysis showed an elevated area under the curve (0.831; 95% CI: 0.634-0.948), with a significance level of p < 0.0014 (area = 0.5). CONCLUSIONS: These preliminary results seem to indicate that breath ethane levels are higher in most patients with ARMD. The breath ethane test could thus be a useful method for evaluating the level of oxidative stress in patients with ARMD. To our knowledge, there are no data on this type of analysis applied to ARMD.

Effect of two-hour daily hemodialysis and sham dialysis on breath isoprene exhalation.

BACKGROUND: Isoprene, a volatile hydrocarbon produced by the human organism, is currently being extensively investigated because the mechanisms underlying its endogenous origin are unknown and because experiments suggest it is toxic and cancerogenous. Previous reports of increases in breath isoprene concentrations during 4-hour, thrice-weekly hemodialysis, but not during continuous ambulatorial peritoneal dialysis, prompted us to assess the behavior of isoprene in another dialytic modality, i.e., short daily hemodialysis (short DHD). Furthermore, in order to determine whether removal of solutes and/or contact of blood with the dialytic membrane influenced the metabolism of isoprene, we performed a sham short hemodialysis session in a subgroup of 8 patients (sham short HD), i.e., with blood flowing through a dialyzer but without dialysate and ultrafiltration. METHODS: The present study evaluates the effects of a two-hour short DHD and a two-hour session of sham HD on isoprene breath levels, as determined by gas chromatography before, during and after sessions. Parallel analyses of ambient air and monitoring of blood pressure and heart rate were performed. RESULTS: Both short DHD and sham DHD induced an increase in breath isoprene exhalation in all patients without being associated with significant hemodynamic variations. CONCLUSION: These findings suggest that the increase in breath isoprene after a session of hemodialysis is neither a reaction to mevalonate depletion nor to metabolic variations induced by the depurative effect, because these changes do not occur during sham HD. It is not related to hemodynamic changes because none were observed in this experimental model. The isoprene increase seems to be of metabolic origin and appears to be connected in some way with the extracorporeal circuit. These interesting findings provide a further impulse to study the biosynthetic pathways involved and to investigate the medical and biological significance of isoprene in humans.

Estimating the incidence of coeliac disease with capture-recapture methods within four geographic areas in Italy.

STUDY OBJECTIVE: To estimate the incidence rate of newly diagnosed cases of coeliac disease in Italy. DESIGN: This was a descriptive study of coeliac disease incidence in the period 1990-91. SETTING: During 1990-91 newly diagnosed cases of coeliac disease were signalled by several sources including diagnostic records of departments of paediatrics, general medicine and gastroenterology, national health service records for the supply of gluten free diets and the archives of the Italian Coeliac Society. PATIENTS: Altogether 1475 cases were flagged throughout Italy, 478 of whom were selected, corresponding to 270 individual patients from a target population resident in four areas: Provices of Turin and Cuneo (Piedmont Region, northern Italy); Province of Brescia (Lombardia Region, northern Italy); Umbria Region (central Italy) and Sardinia Region (insular Italy). Only for these areas were patients flagged from several sources and the reference population was identifiable. MAIN RESULTS: The overall crude incidence rates for all ages per 100,000 residents per year were 2.4, 2.7, 1.5, and 1.7 in the four areas, respectively. The childhood cumulative incidence rates (aged < or = 15 years) per 100,000 live births were 143, 141, 72, and 80 respectively. The mean ages at diagnosis were similar for both childhood and adult cases throughout the areas--these were around 4 and 34 years respectively. For each area, the incidence rate was constantly higher in the main city than elsewhere. Using the capture-recapture method, an estimated completeness of case archives of 0.84 was obtained, whereas this figure was only 0.47 for hospital sources. CONCLUSIONS: This population based study on the incidence of coeliac disease shows that several information sources should be used to avoid underestimation. The incidence rate of coeliac disease in Italy was among the highest in Europe, and was widely variable showing highest figures in Piedmont and Lombardia and the lowest in Umbria and Sardinia. This trend was not due to different age at diagnosis, which suggests variable diagnostic awareness of the disease rather than different environmental patterns affecting the clinical presentation.

Low-amplitude propagated contractile waves: a relevant propulsive mechanism of human colon.

BACKGROUND: Human colonic motility is still poorly understood, especially as far as concerns its propulsive function. Available data refer almost exclusively to the forceful propulsive activity, which is recognized as high-amplitude propagated contractions, the manometric equivalent of mass movements. By contrast, information on less vigorous propulsive contractions is still lacking. AIMS: To investigate the presence and behaviour of low-amplitude propagated contractile waves (less than 50 mmHg in amplitude) in the colon of healthy humans during a 24-hour study period. SUBJECTS AND METHODS: A series of 16 healthy volunteers of both sexes entered the study, and were investigated by a standard technique involving a colonoscopically-positioned manometric catheter. During the study, two standard 1,000 kcal mixed meal and a 450 kcal breakfast were served. The recordings were, therefore, scanned for the presence of low-amplitude propagated contractile waves (waves of less than 50 mmHg in amplitude, propagated over at least three consecutive recording ports), their daily distribution, and their relationship with physiological events. RESULTS: Low-amplitude propagated contractile waves were constantly present in all the tracings, with an average of about 61 events/subject/day and a mean amplitude of about 20 mmHg. More than 80% of these events appeared during the day, with a significant (p<0.05) increase after meals and after morning awakening. In 25% of subjects, these waves were accompanied by emission of flatus. CONCLUSIONS: In the human colon, low-amplitude propagated contractile waves are a constant physiological propulsive pattern, which is generally related to sleep-wake cycles and meal ingestion.

Double-blind manometric assessment of two topical glyceryl trinitrate formulations in patients with chronic anal fissures.

BACKGROUND: Chronic anal fissure is a frequent and troubling condition, that may need surgical sphincterotomy for relief of symptoms. However, this approach may yield minor incontinence in up to 30% of cases. Interest has, therefore, recently increased in "chemical sphincterotomy" by using topical glyceryl trinitrate ointment. Unfortunately, there is, to date, no specific pharmaceutical preparation of such compound. AIMS: To compare, according to a randomized double-blind crossover study, the effects of a pharmaceutical preparation of a specific 0.2% glyceryl trinitrate ointment (PMF 303) and of the common preparation reported in the literature on the anal resting pressure in patients with anal fissure. PATIENTS AND METHODS: Twelve patients with chronic anal fissure (6 males and 6 females, age range 23-60 years] were recruited for the study. Two paired manometric studies were carried out at one-week intervals. After the basal anal pressure had been assessed, the patients were randomized to receive either one of the two preparations, and manometric measurements were repeated at 20, 40 and 60 minutes. RESULTS: No differences were found between anal resting pressure in the basal study. Both preparations were able to significantly decrease (p=0.001) anal pressure throughout the study period. No significant differences were found between the two preparations. CONCLUSIONS: PMF 303 is able to decrease anal pressure in patients with anal fissure, to a similar extent to the widely tested (galenic) literature preparation. Availability of a specific formulation for the treatment of this condition may be clinically useful.

Clinical and manometric aspects of diffuse esophageal spasm in a cohort of subjects evaluated for dysphagia and/or chest pain.

Manometric criteria for diffuse esophageal spasm have recently been restated. In this study, a cohort of 358 subjects was evaluated in a gastrointestinal motility laboratory for dysphagia and/or chest pain. Applying the recently proposed criteria of Richter and Castell, 18 subjects (5%) were diagnosed as having DES. Dysphagia was the major complaint (89%), while 44% of patients complained of chest pain and 33% of both symptoms. All patients shared more than 30% simultaneous contractions after wet swallows interspersed with normal peristaltic sequences. Associated manometric findings were repetitive (greater than 3 peaks) contractions (67%), high-amplitude contractions (33%), spontaneous activity (22%), prolonged duration (11%), and lower esophageal sphincter abnormalities (5%). Radiology disclosed significant abnormalities in only 27% of DES patients.

Valproate-induced epileptic tonic status.

We observed a young patient with slight mental retardation, suffering from drug-resistant tonic-clonic seizures, who presented a status epilepticus (SE), in two separate periods when valproic acid (VPA) was added to the phenobarbital (PB) already being used in the patient's therapy. The VPA-induced SE was characterized by normal plasma levels of antiepileptic drugs (AEDs), normal ammonemia and normal liver function. The case we studied represents the first report on a VPA-induced SE. Furthermore the case confirms that VPA, as well as causing encephalopathy secondary to hyperammonemia, may also provoke a primary involvement of the central nervous system (CNS), specially when used in young epileptic, mentally retarded subjects. In an attempt to explain the paradoxical effect of VPA we hypothesise that the SE could be due to an increase in excitatory activity producing a consequent epileptogenic effect in those subjects with a predisposition for toxic reaction to VPA therapy due to congenital anomalies of neural networks.

Can idiopathic megacolon cause functional motor abnormalities in the upper gastrointestinal tract?

Upper gastrointestinal symptoms are often found in patients with chronic constipation. We report a case of a child with severe chronic constipation due to idiopathic megacolon with megarectum, in which small-bowel motor abnormalities, demonstrated before treatment, were corrected by therapy. Possible pathophysiological mechanisms are discussed.

[Transendoscopic retrograde cholangiography in the diagnosis of primary sclerosing cholangitis. Study of 6 cases]. / La colangiografia retrograda per via transendoscopica nella diagnosi della colangite sclerosante primitiva. Osservazione di sei casi.

Primary sclerosing cholangitis (PSC) is a rare disease of unknown etiology characterized by severe chronic inflammation, fibrosis and stenosis of variable length of the extra and/or intrahepatic biliary ducts in the absence of recent operative trauma, biliary stones, cancer or infection. Diagnosis has been made only at operation. The introduction of endoscopic retrograde cholangiography (ERC) offers the possibility of preoperative diagnosis. Six cases of PSC diagnosed by ERC are presented. Characteristic roentgenologic findings include strictures of variable length of extra and intrahepatic biliary ducts, beaded appearance and decreased arborization of intrahepatic biliary tree. The roentgenological anatomy of biliary tree at ERC influence the subsequent therapy, i.e. surgical therapy is indicated if a drainage can be performed above the site of the stenosis, while medical therapy (steroids and/or immunosuppressive drugs) is the choice when intrahepatic biliary tree is involved.

[Evaluation of transendoscopic retrograde cholangio-pancreatography in 151 jaundiced patients]. / Valutazione della colangio-pancreatografia retrograda per via transendoscopica in 151 pazienti itterici.

Endoscopic retrograde cholangiopancreatography was performed for possible obstruction of the extra-hepatic bile ducts in 151 patients with jaundice. The endoscope findings (9 cases) and the retrograde cholangiopancreatogram (127 cases) provided a final diagnosis in 131 cases. A correct surgical indication was obtaines in a further 5 cases (90% diagnostic success overall). In 19 cases, unnecessary exploratory laparotomy was avoided by the demonstration of normal extrahepatic ducts. No complications were noted. It is concluded that this technique is virtually free from risks and offers prompt diagnosis in subjects with jaundice. This, of course, has a significant influence on the subsequent treatment.

[Reduced ex vivo production of superoxide anion by polymorphonuclear leukocytes during therapy with omeprazole. A pharmacological effect?]. / Ridotta produzione "ex vivo" di anione superossido da parte dei leucociti polimorfonucleati durante terapia con omeprazolo. Un effetto farmacologico?

Among available drugs, omeprazole is the one that cures gastric acid secretion-related pathologies, including reflux oesophagitis which responds poorly to H2-receptor antagonists, most rapidly and efficaciously. This marked therapeutic action is thought to reflect the drug's capacity to adequately control parietal hydrochloric acid secretion. Our data suggest an omeprazole effect on human neutrophil function too. Neutrophils are more or less a constant, and often conspicuous anatomo-pathological component of the phlogistic processes associated with gastric acid secretion. A direct or indirect effect exerted by omeprazole on leukocyte function would be of great scientific-biological and therapeutic interest. Furthermore, it would contribute to marking the drug superior in terms of more rapid relief of the symptoms and range of therapeutic action.

Severe osteomalacia due to gluten-sensitive enteropathy.

This report discusses a severe case of osteomalacia due to gluten-sensitive enteropathy: it stresses the clinical features and describes an atypical form of gluten-sensitive enteropathy, in which gastroenterological symptoms were absent. Wasting and osteomalacia causing skeletal deformation with spontaneous fractures were observed in a 31-year-old woman who had marked hypophosphoremia, a tendency to low serum calcium levels and slight multi-deficiency anaemia. The patient was in a state of depression. The causes of osteomalacia and then a general malabsorption syndrome were investigated. Anti-gliadin antibodies were positive. Histological tests on duodenal mucous revealed a pattern indicative of gluten-sensitive enteropathy. A gluten-free diet was prescribed and at a check-up one month later the patient had improved markedly. Skeletal symptoms are predominant in 30% of atypical forms of gluten-sensitive enteropathy. The severity of this case was due to a late diagnosis.

Hydrogen breath test and scintigraphic gastrocolic transit time in diabetics with autonomic neuropathy.

We assessed gastrocolic transit time in 10 diabetic patients with autonomic neuropathy and 10 healthy age-matched controls by measuring breath hydrogen rise and scintigraphic bolus progression after ingestion of an isosmotic lactulose solution containing 99m-Tc-diethylentriamine-pentaacetic acid. Mean transit time in diabetics with autonomic neuropathy was significantly slower with respect to controls, with a good correlation between the two techniques. Moreover, diabetics had a significantly shorter discharge time (defined as the period that elapses between the arrival of the meal into the cecum and the hydrogen increase in the expired air). It is concluded that the selective lipid malabsorption seen in diabetic patients could be the result of cecal-ileal reflux, as a contaminating consequence of the last ileal loop. A possible motor innervation defect of the ileo-cecal valve is postulated in these subjects.

Functional gut disorders and health care seeking behavior in an Italian non-patient population.

We conducted a survey on functional gut disorders and health care seeking behavior in a large non-patient population of an Italian region (Umbria). 533 subjects were interviewed by means of a specific questionnaire. 44 (8.5%) reported symptoms compatible with the irritable bowel syndrome, 30 (5.8%) had non-colonic pain, 48 (9.2%) chronic constipation, and 20 (3.8%) dyspepsia. It is concluded that in our region there is a relatively high percentage of subjects that do not commonly seek health care, although affected by functional gut disorders.

A double-blind, short-term clinical trial of pirenzepine in duodenal ulcer.

The effectiveness of pirenzepine in the treatment of duodenal ulcer was assessed in a double-blind clinical trial in comparison with placebo. Twenty-nine of 30 patients satisfactorily completed the trial. There was endoscopic ulcer healing in 4 of 14 pirenzepine treated patients (29%) after 2 weeks of treatment and in 11 of 14 (79%) after 6 weeks. There was no healing in 15 patients treated with placebo after 2 weeks and there were two ulcers healed after 6 weeks. The difference between pirenzepine and placebo was significant in both 2nd and 6th week. Duodenitis (evaluated by endoscopy) markedly improved in the pirenzepine grouu after 2 (4 of 9 patients) and after 6 weeks of treatment (8 of 9), while no, or only slight, improvement was observed in 8 of 8 patients in the placebo group. When evaluated by ulcer symptom relief and antacid tablet comsumption, pirenzepine proved to be considerable more efficacious than the placebo. The results of this study demonstrate that pirenzepine promoted duodenal ulcer healing, that it markedly improved peptic duodenitis and promoted rapid regression of symptomatology in parallel with endoscopic findings.

[Chronic therapy with ursodeoxycholic acid in a child with Alagille syndrome]. / Terapia cronica con acido ursodesossicolico in un bambino affetto da Sindrome di Alagille.

The effectiveness of oral bile acid therapy with ursodeoxycholic acid (10 mg/kg/day) was investigated in a 10 year old boy affected by Alagille's Syndrome, a chronic cholestatic disorder due to congenital hypoplasia of the intrahepatic biliary ducts. Cholestatic and hepatonecrotic indices were measured before and during ursodeoxycholic acid therapy and 1 month after stopping and 36 months after restarting the treatment. Ursodeoxycholic acid led to a marked improvement in the cholestatic and hepatonecrotic parameters which was maintained during all the treatment phase. Pruritus and steatorrhea disappeared during the treatment with ursodeoxycholic acid. Histological examination of the liver biopsy after the treatment revealed a disappearance of the biliary plugs but without increasing he intrahepatic bile ducts. The results suggest that ursodeoxycholic acid may improve the condition of the children affected by Alagille's Syndrome, specially when the liver transplantation is required, and indicate a need for long term studies in a larger number of patients.

Prolonged (24-hour) manometric recording of rectal contractile activity in patients with slow transient constipation.

A periodic motor activity, named the rectal motor complex, has been recently described in the healthy human rectum. We studied the rectal contractile activity for 24 h by a low compliance manometric system in a group of 10 women with slow transit constipation. Analysis of the 24-hour manometric recordings showed that these subjects: (1) had overall scarce rectal motility; (2) display few rectal motor complexes (average, 3.3 +/- 1.3/subject/24 h) which are irregularly distributed over time, and (3) respond weakly to ingestion of a standard meal (average duration of the motor response 19 +/- 6 min). The observations suggest that an underlying neuropathic process may be involved in the pathogenesis of the impaired rectal motility in patients with slow transit constipation.