Safety and efficacy of etanercept in children with juvenile-onset Behcets disease.

Behcets disease (BD) is a chronic, relapsing, multi-system inflammatory disorder, clinically characterized by recurrent oral and genital ulcers, skin lesions, and uveitis. Other manifestations include arthritis, a positive pathergy test, thrombophlebitis, central nervous system disease and gastrointestinal ulcerations. The majority of affected individuals do not have life-threatening disease, although mortality can be associated with vascular-thrombotic and neurological manifestations. Currently, treatment of BD is symptomatic and empirical, and is tailored according to the severity of clinical features. In the past few years, isolated reports and case-series have been published on adult BD patients suggesting that inhibition of TNF-alpha is a promising therapeutic approach for severe ocular and various extra-ocular manifestations, including central nervous system involvement. In this study we present our promising experience with Etanercept therapy in juvenile-onset BD patients, characterized by refractory multiorgan involvement.

Consumerism in prenatal diagnosis? A local Italian study.

AIM: To assess the causes of excessive use of prenatal diagnosis. MATERIAL AND METHODS: 304 questionnaires were completed anonymously by puerperae in a Siena (Italy) hospital in May-August 2006. The questionnaires contained 24 questions about the women, examinations performed during pregnancy and the reasons for them. RESULTS: The mean number of ultrasound examinations per woman was 6.5 +/- 2.5. Forty-two percent of the women in our sample (29.3% of women under 35 and 68.9% of women over 35 years of age) reported that amniocentesis/CVS had been performed; the mean age of these women was 34.1 +/- 4.5 years. Eighty-five percent of the women under 36 years of age who had amniocentesis declared that it was performed as a personal choice and 15% for the presence of risk factors. Among 131 women who performed amniocentesis, 32 performed it with a normal blood screening for Down syndrome (DS), and 76 declared to have performed no blood screening for DS. Only 45% of women stated that they thought age above 35 years was a risk factor for pregnancy, but most of them (75%) were aware that amniocentesis was performed to detect chromosomal anomalies. In 89% of the cases a source of information about prenatal testing was the woman's gynecologist. CONCLUSION: This study shows that the high use of prenatal examinations is often not justified by the presence of clinical risk factors and that both national health system and caregivers should find new strategies to inform women about the aims of prenatal tests, and promote a more serene approach to pregnancy. A broader study is needed to confirm these data.

Seizures and electroencephalographic findings in CDKL5 mutations: case report and review.

Mutations in the X-linked gene cyclin-dependent kinase-like 5 (CDKL5) have been detected in patients presenting with seizures in the first few months of life and Rett syndrome features. Twenty-seven cases have been detected to date. Generalized intractable seizures, as infantile spasms, and generalized tonic-clonic seizures and myoclonic seizures characterize the clinical picture of CDKL5 mutations. Here we report on a patient who presented with sleep-related hyperkinetic seizures. Our observation and review of the literature suggest that a broader polymorphic electroclinical pattern with both generalized and focal seizures may occur in patients with CDKL5 mutations. A screen for CDKL5 mutations is useful in patients, mainly females, with a history of early onset intractable seizures and becomes mandatory when idiopathic infantile spasms and/or atypical Rett syndrome features are also present.

High-Dose Robotic Stereotactic Body Radiotherapy in the Treatment of Patients With Prostate Cancer: Preliminary Results in 26 Patients.

BACKGROUND: Stereotactic body radiotherapy (SBRT) can emulate high dose rate brachytherapy (HDR-BRT) dose fractionation. We report our preliminary results using SBRT in monotherapy or pre-external-beam radiotherapy (EBRT) boost in patients with localized prostate cancer (LpC). The primary end point was the evaluation of both acute and late toxicities; secondary end point was the observation of prostate-specific antigen (PSA) nadir. PATIENTS AND METHODS: Patients with LpC having prostate volume ≤90 cm(3) were enrolled in the present study. Patients were treated with SBRT alone or in combined modality (SBRT + EBRT). SBRT was performed using a CyberKnife System (Accuray Incorporated, Sunnyvale, California) and fiducial tracking system. RESULTS: From February 2008 to July 2013, 21 patients for monotherapy (38 Gy/4 fractions) and 5 for combined modality (9.5 Gy/2 fractions plus 46 Gy/23 fractions EBRT) were enrolled. Androgen deprivation therapy (ADT) was administered in 16 of the 26 patients. The median pretreatment PSA was 9.4 (range, 4.5-14.3) ng/mL. All patients completed the planned therapy. Acute Grade 1 toxicity was observed in 18 patients, genitourinary (GU) in 12 / 26 patients, and gastrointestinal (GI) in 6 / 26 patients. Acute Grade 2 GU toxicity was reported in 1 / 26 patients, and Grade 2 GI toxicity was observed in 2 / 26 patients. The median PSA nadir was 0.15 (range, 0.02 = 1.4) ng/mL. Late toxicities were observed in 5 / 26 patients: Grade 1 GU (3 of 26), Grade 2 GU (1 of 26), and Grade 1 GI (1 of 26). Median follow-up was 21.5 (range, 8-65) months. CONCLUSIONS: Our preliminary results of SBRT "simulating" HDR for LpC confirm a minimal toxicity and an optimal PSA response. The PSA nadirs appear comparable with HDR-BRT.

Autosomal recessive hypercholesterolemia in a Sicilian kindred harboring the 432insA mutation of the ARH gene.

We describe a Sicilian family presenting a recessive form of hypercholesterolemia harboring a mutation of the autosomal recessive hypercholesterolemia (ARH) gene. In two of the three sibs, a 26-year-old male and a 22-year-old female, a severe hypercholesterolemia was diagnosed with very high levels of plasma cholesterol (15.9 and 12.2 mmol/l, respectively); tendon xanthomatas and xanthelasms were present and in the male proband was documented a diffuse coronary atherosclerotic disease with a rapid and fatal progression. Both the parents had normal or slightly increased levels of plasma cholesterol. All causes of secondary hypercholesterolemia were ruled out as well as an involvement of the LDL receptor or apoB genes. Beta-Sitosterol plasma levels were in the normal range. Cultured fibroblasts from skin biopsy from parents and the two probands displayed a normal ability to bind and degrade 125I-LDL. Direct sequencing of ARH gene demonstrated the presence of a 432insA mutation in homozygosis in the two probands; parents were heterozygotes for the same mutation. This mutation is the first report of a mutation of the ARH gene responsible for recessive forms of hypercholesterolemia in Sicily.

Ranitidine bismuth citrate versus omeprazole triple therapy for the eradication of Helicobacter pylori and healing of duodenal ulcer.

AIM: To compare the efficacy and safety of triple therapy with omeprazole plus amoxycillin and clarithromycin vs. ranitidine bismuth citrate plus amoxycillin and clarithromycin in the treatment of Helicobacter pylori-associated duodenal ulcers. METHODS: Eighty-one patients with duodenal ulcers were randomized to the following treatments: 39 cases with amoxycillin 1 g b.d. and clarithromycin 500 mg b.d. for 1 week plus omeprazole 20 mg b.d. for 2 weeks (omeprazole + amoxycillin + clarithromycin (OAC)), and 42 cases to the same regimen of amoxycillin and clarithromycin for 7 days plus ranitidine bismuth citrate 400 mg b.d. for 2 weeks (ranitidine bismuth citrate + amoxycillin + clarithromycin (RbAC)). Upper gastrointestinal endoscopy was performed together with a rapid urease test and histological examination of antral and corpus biopsy samples prior to treatment and 4 weeks after the end of therapy. RESULTS: Thirty-four patients in the OAC group and 38 in the RbAC group completed the treatment and 4-week follow-up. H. pylori was eradicated in 30 of 34 patients (88%) in the OAC group and in 32 of 38 patients (84%) in the RbAC group according to a per-protocol analysis (P = N.S.). Thirty-three (97%) patients treated with OAC and 36 (95%) treated with RbAC presented healed duodenal ulcers at 4 weeks (P = N.S.). On an intention-to-treat basis there was no difference in H. pylori eradication between the OAC (77%) and RbAC groups (76%); duodenal ulcer healing was achieved in 85 and 86% of patients in the OAC and RbAC groups, respectively (P = N.S.). CONCLUSION: The OAC and RbAC triple therapy regimens proved equally effective in both H. pylori eradication and in duodenal ulcer healing.

Helicobacter pylori-positive duodenal ulcer: three-day antibiotic eradication regimen.

BACKGROUND: The most widely used treatments for ulcer healing and Helicobacter pylori eradication consist of a 1-2 week regimen of a proton pump inhibitor plus two or three antimicrobials. AIMS: To evaluate the efficacy, safety, cost, and tolerance of a three-day regimen with three antibiotics vs. a 10-day treatment with a proton pump inhibitor or vs. a ranitidine bismuth citrate triple therapy. METHODS: Two hundred and twenty-one patients with endoscopically-proven H. pylori-positive duodenal ulcers were recruited to the study. Recruited patients were assigned to one of the following four regimens: (I) omeprazole 40 mg o.m. plus amoxycillin 1 g b.d. and clarithromycin 500 mg b.d. for 10 days (OAC: 55 patients); (ii) omeprazole 40 mg o.m. on days 1-5, plus amoxycillin 1 g b.d., clarithromycin 500 mg b.d. and metronidazole 500 mg b.d. on days 3-5 (OACM: 56 patients); (iii) ranitidine bismuth citrate 400 mg b.d. plus amoxycillin 1 g b.d. and clarithromycin 500 mg b.d. for 10 days (RAC: 54 patients); (iv) ranitidine bismuth citrate 400 mg b.d. on days 1-5, plus amoxycillin 1 g b.d., clarithromycin 500 mg b.d. and metronidazole 500 mg b.d. on days 3-5 (RACM: 56 patients). Fisher's exact test was used to compare data regarding healing and eradication in the four groups. RESULTS: The intention-to-treat eradication and ulcer healing rates for the RACM regimen were 95% and 98%, respectively. Statistically significant differences were observed, relating to the eradication and healing of ulcers, between RACM and either the RAC or OAC regimens. CONCLUSION: The three-day antibiotic therapy with amoxycillin, clarithromycin and metronidazole in addition to ranitidine bismuth citrate is a very effective anti-H. pylori regimen.

Ultrasound demonstration of gallbladder wall thickening as a method to differentiate cirrhotic ascites from other ascites.

RATIONALE AND OBJECTIVES: Gallbladder wall thickness was measured by ultrasound in 16 patients with ascites caused by liver cirrhosis and in 16 noncirrhotic patients with ascites. This study was performed to evaluate if gallbladder wall thickening could differentiate cirrhotic ascites from ascites due to other causes. METHODS: Gallbladder thickness was measured by duplex ultrasound in 16 cirrhotic patients with ascites and in 16 noncirrhotic patients with ascites. Measurements of portal vein flow and serum albumin also were performed. RESULTS: Gallbladder wall thickness was 0.76 +/- 0.21 cm in cirrhotic patients and 0.24 +/- 0.09 in noncirrhotic patients (P < 0.001, Student's t test for unpaired data). Gallbladder wall thickening was significantly more frequent in patients with cirrhotic ascites than in patients with noncirrhotic ascites (P < 0.001, chi-square test). CONCLUSIONS: The authors feel that the ultrasound finding of gallbladder wall thickening in patients with ascites is highly predictive of liver cirrhosis diagnosis.

Influence of aging on gastrointestinal transit time. An ultrasonographic and radiologic study.

RATIONALE AND OBJECTIVES: To assess and compare gastrointestinal (GI) rates in young and aged men under homogeneous conditions of weight and dietetic habits. METHODS: Gastric emptying time was evaluated by ultrasound. GI transit time was studied radiologically using radiopaque markers. RESULTS: Final gastric emptying time in elderly subjects was 335 +/- 31 minutes (mean +/- SD) versus 245 +/- 25 minutes in young subjects (P < 0.001). Total GI transit time showed no significant difference between the two groups. Intestinal transit time includes both small bowel and colonic transit; small bowel and colonic transit was not separated in this study. CONCLUSIONS: Delayed gastric emptying of solid foods could result from progressive autonomic nerve dysfunction occurring with aging. The stomach does not seem to be the segment of the digestive tract that is primarily responsible for the alteration of total GI time.

Gastric emptying rates of solid food in relation to body mass index: an ultrasonographic and scintigraphic study.

Gastric emptying can be modified by different physiologic conditions such as aging, menstrual cycle and pregnancy. Few studies in the literature have compared the gastric emptying rate of solid meals in normal subjects of varying size. The purpose of this work is to evaluate the gastric emptying rate of solid meals to determine whether body mass index (BMI) and gastric emptying rate correlate. Both ultrasonographic and scintigraphic techniques have been employed. Twenty-four healthy male subjects, divided into two groups, participated in the study. Our results demonstrate a significant correlation between gastric emptying and BMI. We conclude that variability of BMI must be taken in account when measurements of gastric emptying of solid food are performed.

[Gastric emptying of solids in patients with nonorganic dyspepsia]. / Vuotamento gastrico dei solidi in pazienti con dispepsia anorganica.

The purpose of this work was to evaluate gastric emptying of a solid meal in 20 patients with functional dyspepsia and in 20 healthy controls. Gastric emptying time (T 1/2 = 188.45 +/- 93.79; p less than 0.001 - E.I. = 0.43 +/- 0.16; p less than 0.001) was significantly delayed in patients with functional dyspepsia compared with controls. The conclusion is drawn that either antral motor activity or antral-pyloric-duodenal motor activity is impaired in such patients.

Gallbladder benign neoplasms: relationship with lithiasis and cancer (ultrasonographic study).

BACKGROUND: The aim of this study is to clarify the prevalence of gallbladder benign neoplasms, their ultrasonographic appearance and their relationship with gallbladder lithiasis and cancer. METHODS: This study was carried out on 9000 consecutive patients having ultrasound of upper abdomen. Only adenomas and papillomas are considered as true benign neoplasms of the gallbladder. Adenomiomatosis and cholesterol polyps, often erroneously labelled as benign neoplasms, were excluded. Patients were followed-up by ultrasound every three months up to two years. RESULTS: The prevalence of benign neoplasms was 1.19%. Papillomas were found more frequently than adenomas both in males (68.51%) and in females (94.33%). Gallstones were not concomitant with benign neoplasms in any case. Neither stones nor growth of gallbladder benign neoplasms were recorded within the two-year follow-up period. CONCLUSIONS: Papillomas were more frequent than adenomas. No gallstone was concurrent with gallbladder benign neoplasms in our series. However, when gallstones are evidenced at ultrasound, further attention is recommended to discover probable concomitant neoplasms. Papillomas and adenomas more than 1 cm in diameter should be quarterly followed-up, while smaller masses could be six-monthly controlled. Surgery should be indicated for large-sized or rapidly growing masses because of the risk for cancer development.

[Levosulpiride versus domperidone in the treatment of functional dyspepsia]. / Levosulpiride vs domperidone nel trattamento della dispepsia funzionale.

Levosulpiride, a dopaminergic antagonist was compared in a double-blind randomized study with domperidone for treatment of functional dyspepsia in 50 patients divided into two groups of 25 subjects each. Group I received 25 mg levosulpiride 3 times daily, group II received 10 mg domperidone 3 times daily, all for 30 days. Periodic clinical examination at days 0, 7, 15, 30 showed symptomatic changes. Gallbladder and gastric emptying was studied by ultrasonography at the start and end of treatment. Both drugs had a positive influence on dyspeptic symptoms and on gastric and gallbladder emptying, but the latter parameters were improved significantly more effectively by levosulpiride. As to tolerability, there have been 3 drop-outs and a further 6 patients complained of sides effects that did not require suspension of treatment.

Common bile duct caliber following cholecystectomy: a two-year sonographic survey.

The aim of this study was to evaluate the size of the common bile duct (CBD) in patients with gallstones, before and after cholecystectomy. Ten female patients (22-61 years) were examined in whom gallstones were shown by ultrasound. The caliber of CBD was measured before cholecystectomy and then 6, 12, 18, and 24 months after cholecystectomy. No significant change of CBD caliber in each interval prior to or following cholecystectomy was observed.

Sonographic study of postprandial gallbladder emptying and common bile duct changes in patients with diabetes or cholelithiasis.

The aim of our study was to evaluate postprandial emptying of the gallbladder and common bile duct (CBD) in 20 patients with gallstones and in 20 diabetic patients with or without autonomic neuropathy. Gallbladder fasting volume was significantly increased in patients with multiple stones. Diabetics with autonomic neuropathy showed a decreased gallbladder emptying rate. CBD size did not show any significant change in all groups examined.

Eradication therapy with rabeprazole versus omeprazole in the treatment of active duodenal ulcer.

BACKGROUND: Rabeprazole has been demonstrated to be a potent antisecretory agent and has been shown to be clinically effective in the treatment of acid-related diseases. AIMS: It was to determine the efficacy of rabeprazole at 20 and 40 mg in addition to amoxicillin and clarithromycin in the treatment of active Helicobacter pylori-positive duodenal ulcers compared with omeprazole 40 mg. PATIENTS AND METHODS: One hundred and twenty-seven patients were randomised into three treatment groups: 40 patients were treated with rabeprazole 40 mg daily, 42 patients with rabeprazole 20 mg daily and 45 patients with omeprazole 40 mg daily for 10 days. All patients received amoxicillin 1 g twice a day and clarithromycin 500 mg twice a day for 5 days. All patients were re-assessed at least 4 weeks after the end of the treatment. RESULTS: According to the intention-to-treat (ITT) protocol, ulcer healing was observed in 90% of patients in the rabeprazole 40 group, in 85.7% in the rabeprazole 20 group and in 93.3% in the omeprazole 40 group. We observed H. pylori eradication in 90% ITT in the rabeprazole 40 group, in 80.9% ITT in the rabeprazole 20 group and in 88.8% ITT in the omeprazole 40 group. Statistical analysis did not show significant differences among the three groups. CONCLUSIONS: A 10-day rabeprazole 20 mg regimen represents an efficacious and safe regimen for H. pylori eradication and ulcer healing.

Gastric clearance of radiopaque markers in the evaluation of gastric emptying rate.

BACKGROUND: The study of gastric emptying rate of solids using radiopaque indigestible solid markers has been a poorly employed technique because some kinds of markers do not leave the stomach at the same time as the meal but during the interdigestive migrating motor complex (IMMC). The aim of this study was to evaluate whether markers of particular shape and size can be successfully employed for this purpose. METHODS: Twenty-eight non-ulcer dyspeptic (NUD) patients and 20 healthy volunteers received a standard solid meal (790 Kcal) together with 20 small polyethylene radiopaque cylinders (5 mm x 2 mm in diameter). Gastric emptying rate was evaluated by ultrasound while the emptying of markers was simultaneously followed by X-rays using a brilliance intensifier. RESULTS: Final emptying time (FET = time when the antrum area returns to fasting size) of digestible solids was 355+/-35 min in NUD patients versus 265+/-20 min in controls (P < 0.001). The gastric emptying curve of digestible solids correlated with emptying of markers both in NUD patients (r= +0.96) and in controls (r= +0.93). CONCLUSIONS: The assessment of gastric clearance of radiopaque cylinders of 2 mm x 5 mm in size is a reliable tool for the study of gastric emptying rate of digestible solids. This is a readily available and easily performed test in any radiology unit.

Copper (II) complexes of aminoacids: gastric acid antisecretory activity in rats.

The effect of simple and mixed copper(II) complexes of L-tryptophan (trp) and L-phenylalanine (phe) on the gastric acid secretion was investigated in Shay rats. Graded doses (from 15 to 75 mg/kg) of [Cu(trp)2(H2O)], [Cu(phe)2(H2O)] and [Cu(trp)(phe)(H2O)], administered i.g., caused a progressive inhibition of gastric acid secretion. The antisecretory activity of the mixed complex, [Cu(trp)(phe)(H2O)], was significantly greater (ID50 = 33 +/- 3 mg/kg) than that of the simple complexes [Cu(trp)2(H2O)] (ID50 = 48 +/- 3 mg/kg) and [Cu(phe)2(H2O)] (ID50 = 43 +/- 3 mg/kg). At the experimental conditions of intragastric pH, the different biological activities were directly related to the percentage of neutral species present in solution as determined by means of potentiometric measurements (at 37 degrees, I = 0.15 mol dm-3 in NaClO4). A possible mechanism of action of the investigated compounds is discussed.