["A minute for life": resuscitation techniques in infants with risk for sudden infant death syndrome. Development of an instructional video for parents]. / "Un minuto per la vita": tecniche rianimatorie in lattanti a rischio di morte improvvisa. Realizzazione di un video didattico per i genitori.

AIM: The present study describes a project carried out in the Center for SIDS/ALTE of the Pediatric Clinic of Varese, targeted to deliver and provide parents with a movies on PBLS titled "A Minute for Life". The impact on the parent was evaluated by applying a questionnaire. METHODS: The movie "A Minute for Life" was given to 308 parents (122 fathers, mean age 35.2 and 186 mothers, mean age 28.4). All parents completed a questionnaire based on 4 parameters: 1. clarity of the content presented; 2. anxiety felt while watching the movie clip 3. reassurance related to their ability to review other times the movie; 4. the perception of its usefulness. RESULTS: Regarding the clarity of content, the results provide evidence that 231 parents (75% of the sample) rated him "very clear", while 77 subjects (25%) considered it "quite clear". On the possibility of being able to have at home, 277 parents (90% of subjects) believed it would be very reassuring to see it back Home. According to 231 parents (75% of the sample) the vision of the movie does not convey anxiety, while 77 of them (25% of subjects) felt slightly concerned in relation to vision. With regard to the overall assessment of the movie, all parents (308, 100% of the sample) agreed about its extreme usefulness. CONCLUSION: Our study opens the way for further prospective studies regarding the appropriateness and usefulness of the movie. It's also important to consider the release of the movie to all new parents and the possibility of giving first aid courses open to all those who want to be able to act with promptness and expertise if and when necessary.

Down syndrome, autoimmunity and T regulatory cells.

Autoimmune diseases are more represented in Down syndrome (DS) individuals compared to chromosomally normal people. Natural T regulatory cells (nT(reg) ) have been considered to be primary in the role of controlling the intensity and targets of the immune response. We have investigated the phenotypical and functional alteration of nT(reg) in a group of DS people. The phenotypical characteristic of T(reg) cells of 29 DS was analysed and compared with an age-matched healthy control group. The inhibitory potential of CD4(+) CD25(high) CD127(low) T regulatory cells was evaluated on autologous CD4(+) CD25(-) T cell proliferation in response to activation with a mytogenic pan-stimulus (anti-CD2, anti-CD3 and anti-CD28 antibodies). The CD4(+) CD25(high) cells in the DS and control groups were 2·692±0·3808%, n=29 and 1·246±0·119, n=29%, respectively (P=0.0007), with a percentage of forkhead box protein 3 (FoxP3)-expressing cells of 79·21±3·376%, n=29 and 59·75±4·496%, respectively (P=0.0015). CD4(+) CD25(+) FoxP3(+) cells were increased in peripheral blood from DS subjects (DS mean 5·231±0·6065% n=29, control mean 3·076±0·3140% n=29). The majority of CD4(+) CD25(high) were CD127(low) and expressed a high percentage of FoxP3 (natural T(reg) phenotype). While the proliferative capacity of DS T cells was not altered significantly compared to normal individuals, a reduced inhibitory potential of T(reg) compared to healthy controls was clearly observed (mean healthy control inhibition in T(eff) : T(reg) 1:1 co-culture: 58·9%±4·157%, n=10 versus mean DS inhibition in T(eff) :T(reg) 1:1 co-culture: 39·8±4·788%, n=10, P=0.0075; mean healthy control inhibition in T(eff) : T(reg) 1:0·5 co-culture: 45·10±5·858%, n=10 versus DS inhibition in T(eff) : T(reg) 1:0·5 co-culture: 24·10±5·517%, n=10, P=0.0177). DS people present an over-expressed peripheral nT(reg) population with a defective inhibitory activity that may partially explain the increased frequency of autoimmune disease.

Short-term effects of growth hormone treatment on the upper airways of non severely obese children with Prader-Willi syndrome.

AIMS: The aim of this study was to establish whether short-term GH treatment causes obstructive apnea in patients with Prader-Willi syndrome and normal upper airway patency. SUBJECTS AND METHODS: We performed an observational longitudinal 6-week GH treatment study. Thirty-four non-severely obese Prader-Willi syndrome patients (20 boys, age range 0.94-11.8 yr, median 2.24 yr) entered an observational longitudinal 6-week study. Sixteen boys received recombinant human GH (rhGH) treatment; the remaining 18 represented the control group and received no treatment. Polysomnography monitoring and othorhinolaringoiatric video endoscopy were performed one night before and after 6 weeks of rhGH treatment (0.03 mg/kg body weight/day). All patients underwent auxologic assessment, fasting blood glucose, insulin and IGF-I evaluation. The main polysomnographic parameter considered was total apnea hypopnea index, consisting of two components: central apnea hypopnea index and obstructive apnea hypopnea index. All patients were free of severe or moderate upper airway obstruction when rhGH treatment began. RESULTS: After 6 weeks of rhGH therapy, obstructive apnea hypopnea index increased in 8/16 (50%), decreased in 5/16 (31%), and did not change in 3/16 (19%) patients. The changes were not statistically significant. The rhGH-treated group did not differ from the control group for the apnea hypopnea index both before and after 6 weeks of treatment. Adenoids and tonsils showed a slight increase in 1 and 2 patients on rhGH treatment, respectively, and did not change in the untreated patients. CONCLUSIONS: Our data show that short-term rhGH treatment does not cause restrictions of the upper airways in patients with Prader-Willi syndrome and normal upper airway patency.

Phenotype and function of dendritic cells and T-lymphocyte polarization in the human colonic mucosa and adenocarcinoma.

AIM: To evaluate the status of activation of the intestinal dendritic cells (DCs) and T lymphocytes (T cells) from surgical specimens of human colon and adenocarcinoma, and the potential effect of administration of interleukin 2 (IL-2). METHODS: Patients undergoing colectomy for cancer were randomized to receive subcutaneous IL-2 (12million UI/day) (treated group; n=10) for 3days before operation or no treatment (control group, n=10). DCs and T cells were isolated and purified from the lamina propria (LP) of segments of normal colon and adenocarcinoma of both groups. Cell phenotype was determined by expression of membrane receptors. Interaction between DC and T cells was assesses by a mixed leukocyte reaction using naïve T cells co-cultured with DCs. CD4+ T-cell polarization was studied by intracellular staining with monoclonal antibodies for interleukin-4 and interferon-gamma. RESULTS: CD4+ T cells were significantly less in tumour than in LP (p<0.05) in both treated and control groups. IL-2 did not modify the number of any of the T-cell subsets analysed. In contrast, T cells isolated from LP and neoplasm of treated patients produced more interferon-gamma and less interleukin-4 (p<0.05 vs. controls). IL-2 administration significantly increased (p<0.05) the number of mature, myeloid and plasmocytoid DCs compared to controls. Allogeneic naïve T cells were polarized toward a Th1 type of response which appeared to be mediated by IL-2 activated DCs. CONCLUSIONS: systemic IL-2 treatment may have immunomodulatory properties on intestinal DC maturation and drive a Th1 mediated anti-neoplastic response.

[Probiotics, prebiotics and zinc in the therapy and prevention of acute infectious diarrhoea in children: state of the art]. / Probiotici, prebiotici e zinco nella terapia e prevenzione della diarrea acuta infettiva in età pediatrica: qual' è l'attuale evidenza?

Selected probiotics (mainly Lactobacilli, and particularly LGG, and Saccharomyces boulardii) have recently demonstrated a therapeutic efficacy in acute diarrhoea, if used in the early phase of infection and at high concentration. Further data are needed to clarify their effect for prevention and travellers' diarrhoea. The mechanisms of action of probiotics need to be fully elucidated but seem to include a complex interaction of epithelial, molecular, metabolic and immune responses. There is an increasing evidence that different micro-organisms show different properties and efficacy. An accurate identification and selection of the strains, the dose and the patients are thus crucial for a correct therapeutic approach. Prebiotics can modify the intestinal flora and interact with the immune system of the host against specific pathogens. However, clinical trials are currently limited and a beneficial effect of prebiotics in acute diarrhoea is still lacking. In developing countries zinc supplementation demonstrated a significant reduction of fecal excretion, duration, severity and persistency of diarrhoea. Moreover, zinc may improve immune status, intestinal permeability, epithelial and enzymatic functions, and transport of electrolytes. The use of zinc in addition to oral rehydration solution (ORS) could thus theoretically improve the treatment and reduce the complications of diarrhoea worldwide. However, in developed countries, no trial using zinc supplementation in patients with acute diarrhoea has been published yet and the cost-benefit ratio of zinc supplementation needs to be assessed.

Multiple sclerosis and celiac disease: is there an increased risk?

Multiple sclerosis and celiac disease are both considered immune-mediated diseases. Recently, improved serological screening methods provided a higher prevalence of celiac disease (CD) in the general population worldwide and also demonstrated gastrointestinal symptoms may be lacking. The aim of this study was to determine the prevalence of (CD) in an unselected group of 95 adults with multiple sclerosis using transglutaminase antibodies. No patients showed pathological values. Different immune and genetic basis between the two diseases may represent crucial insights to explain our results.

Late diagnosis in severe and mild intellectual disability in adulthood.

BACKGROUND: The diagnosis of intellectual disability (ID) is highly dependent on a comprehensive personal and family medical history, a complete physical examination and a careful developmental assessment of the patient. Our study intended to: (1) classify the aetiology of mild and severe ID in an adult population of 140 Italian subjects; (2) evaluate the frequency of associated medical conditions; (3) evaluate the age of diagnosis in both groups; and (4) underline the importance of aetiological diagnosis for adult ID patients also. METHODS: The study involved 140 consecutive adult Italian ID inpatients and outpatients neurologically investigated at the Neurological Institute C. Mondino of Pavia Service for Mental Retardation. A total of 80 patients had mild ID (MID group) (39 females, 41 males), mean age 34 years (range 19-61 years), mean IQ = 64 (range 51-75), and 60 had severe ID (SID group) (32 females, 28 males), mean age 30 years (range 19-69 years). They underwent a complete diagnostic work-up that comprised prenatal, perinatal and postnatal history, physical examinations, laboratory investigations, genetic survey and neuroradiological investigations to determine the aetiology of ID and to evaluate the presence of associated medical conditions. RESULTS: ID aetiology was classified as prenatal in 34% of the MID and 28% of the SID group. Perinatal and postnatal events were found in 6% of the MID and in 5% of the SID group. Associated medical conditions were found in 97 patients (47% MID and 26% SID). A genetic diagnosis was possible in 6% of patients above 20 years of age and in 5% of patients above 40 years. A diagnosis of cerebral dysgenesis was possible in 5% of patients above 20 years and 4% of patients above 40 years. CONCLUSIONS: A long interval between the diagnosis of ID and the aetiological definition can be observed in a significant percentage (24%) of our population, leading to unfortunate consequences of late diagnosis: late onset of a specific therapeutic program, genetic counselling that is frequently no more useful, and ineffective prenatal diagnosis, leading to the birth of other affected subjects (for familiar ID).

[Clinical role of interleukin-2 in the surgical treatment of liver metastasis due to colon adenocarcinoma]. / Ruolo clinico dell'interleuchina-2 nel trattamento chirurgico delle metastasi epatiche da adenocarcinoma del colon.

The surgical treatment of liver metastasis due to colorectal cancer can substantially modify the natural history of the disease, mainly when it is associated with effective medical treatment. Chemotherapy, via systemic or locoregional (intrahepatic) administration, has 2 possible objectives: as adjuvant treatment, to prevent or delay disease recurrence; as neo-adjuvant treatment, mainly interesting for the surgeon, to allow resective surgery in responding patients previously considered not-operable. Unfortunately, the severe immune deficiency associated with the advanced cancer negative impact on long-term outcome after any treatment (surgery, chemotherapy) is a limit for the clinical application of multidisciplinary treatments. Aim of this study is to review the possible different approaches to improve the clinical results, either as tumour response or overall survival, using an association of IL-2 with different chemotherapy procedures, in order to recover the locoregional and/or systemic immunodeficency. Several literature studies are worth of consideration not only for the biological activity reported, but also for the preliminary clinical results. At our Department, we have started a clinical experience in order to verify and confirm the results reported in these studies. The preliminary results seem to confirm an increase of chemotherapy activity obtained with an association of IL-2 immunotherapy with systemic therapy procedures and mainly with locoregional therapeutic programs.

Silent myocardial ischemia in diabetic and nondiabetic patients with coronary artery disease.

BACKGROUND: Patients with diabetes mellitus are at increased risk for CAD; silent ischemia is reported to be frequent in diabetic populations. The aim of the present study was to evaluate the prevalence of silent ischemia in diabetic and nondiabetic patients with assessed CAD. METHODS AND RESULTS: We recruited a total of 618 patients with CAD: 309 were consecutive diabetic patients and 309 were age- and gender-matched nondiabetic patients. Myocardial ischemia was evaluated both during daily life and during exercise testing. Angina pectoris during daily life was more frequent in diabetic than in nondiabetic patients (80% vs. 74%, P<0.05). The anginal pain intensity either during daily life or acute myocardial infarction (MI), the prevalence of a previous MI, the extent of CAD and ergometric parameters were similar in diabetics and nondiabetics. Silent ischemia during exercise was documented in 179 (58%) diabetics and in 197 (64%) nondiabetics (nonsignificant, ns). Both diabetics and nondiabetics with silent exertional myocardial ischemia differed from symptomatic subjects in higher heart rate values (P<0.01), systolic blood pressure (P<0.01), rate-pressure product (P<0.001), work load (P<0.01) and maximum ST-segment depression at peak exercise (P<0.05). CONCLUSIONS: The incidence of silent myocardial ischemia during exercise was similar in diabetic and nondiabetic CAD patients. Surprisingly, diabetics showed a higher prevalence of angina pectoris during daily activity than nondiabetics. A significant association between the presence of symptoms during daily life and exercise was observed in both groups. Our results may contribute to the planning of the clinical management of diabetic CAD patients and confirm the individual attitude to pain of CAD patients independent of the presence of diabetes.

[Nutritional therapy in surgical patients: an update]. / Terapia nutrizionale nei pazienti chirurgici. Un aggiornamento.

Patients undergoing major gastrointestinal surgery, often require an adequate artificial nutritional (AN) support for a pre-existing state of malnutrition and/or to overcome forced periods of postoperative starvation and/or for complications that alter the host metabolic response. When an indication to AN is given, enteral feeding should be preferred to parenteral nutrition because more physiological and less expensive. Moreover, recent data showed that patients fed enterally, rather than parenterally, in the postoperative period, have a significant better outcome with a reduction of morbidity and hospitalisation. The supplementation of standard feeds with key nutrients having immunomodulatory properties, such as arginine, omega-3 fatty acids and glutamine (pharmaconutrients), allows to control effectively the surgery-induced immunosuppression and hyperinflammation. An analysis on the principles of evidence-based medicine, supports the hypothesis that the pre-perioperative use of formulas enriched with pharmaconutrients, significantly reduces the rate of infectious complications and saves health care resources.

[Spontaneous pneumothorax caused by lung metastasis of sarcoma of the thigh]. / Pneumotorace spontaneo da metastasi polmonare di sarcoma della coscia.

Relapsing spontaneous pneumothorax can be the first manifestation of pulmonary metastases of soft tissues sarcomas. Standard imaging techniques and computed tomography may not be able to detect small malignant cystic lesion or to distinguish between them and benign bollous lesions. We report the case of a 33 year-old male who, in the past, underwent surgical treatment for a synovial sarcoma of the inferior limb. The patient was admitted to our hospital because of right spontaneous pneumothorax; both chest x-ray and CT scan didn't detect any metastatic pulmonary lesion. A few days after the discharge the patient was readmitted because of relapsed pneumothorax; high-definition CT of the chest revealed a pulmonary cystic lesion that was resected thoracoscopically. Histological examination revealed a pulmonary metastases of synovial sarcoma.

Body composition and growth in asthmatic children treated with inhaled steroids.

BACKGROUND: Prolonged treatment with inhaled steroids is recommended for long-term control of asthma in children; however, it can interfere with growth and body composition. OBJECTIVE: The aim of this study is to answer the question whether 6 months treatment with inhaled steroids causes body fat accumulation and growth velocity reduction. METHODS: Hospital-based, open study of body composition [by dual-energy X-ray absorptiometry (DXA), bioelectrical impedance analysis (BIA) and skinfolds] and growth of 26 asthmatic children, treated for 6 months with inhaled steroids [budesonide (BUD) 400 microg/day (group 1) or fluticasone proprionate (FP) 200 microg/day (group 2)], sodium cromoglycate and beta2-agonist (salbutamol) compared with a control group of 16 asthmatic children treated only with sodium cromoglycate and beta2-agonist. RESULTS: On average, total and regional fat mass, adjusted for pubertal stage and gender, and growth velocity were similar in all three groups of patients and were not influenced by treatment (% mean change +/- 1 SD of fat mass during treatment in BUD 0.1 +/- 3.0%, FP -1.1 +/- 3%, and control -2.8 +/- 3.5%; ANOVA P > or = .05); however seven patients, two in group 1 (1 preschool child), three in group 2 (2 preschool children) and two in the control group (two prepubertal boys aged 8.5 and 9.5 year), during treatment, showed a growth velocity standard deviation score below the third percentile. CONCLUSION: A 6-month treatment with inhaled BUD and FP does not induce body fat accumulation; however, in a few preschool children the treatment was associated with growth velocity below the third percentile. Our results suggest the need for constant monitoring of growth in all asthmatic children on chronic treatment with inhaled steroids. Further studies devoted to the effects of inhaled steroids use in preschool children are needed.

The management of shock and local injury in traumatic rhabdomyolysis.

Rhabdomyolysis (literally "striped muscle dissolution") is a biological and clinical condition that takes to plasmatic release of myoglobin, muscle enzymes and electrolytes, relates to the lysis of stripped muscle fibers. Rhabdomyolysis presents the clinician with two distinct problems: local injury and the systemic effects directly related to that injury. Locally, muscle, vessel and nerve compression are the primary issues. Systemic concerns relate to depleted intravascular volume, electrolyte imbalances and renal injury from myoglobin. Preventing the systemic and renal complications of the crush syndrome requires very early and vigorous treatment to sustain the circulation, preferably started at the site of the catastrophe. During the extrication of an injured person from a collapsed building, wrecked automobile, or other site, isotonic saline solution should be infused at the rate of 1.5 liters per hour as soon one of the trapped person's limbs has been freed. Some authors suggest to do a preventive fasciotomy in any suspicious case of compartmental syndrome, when the patient has severe muscular pain of the muscular cavity, tense swelling, hypoesthesia or anesthesia of the muscular cavity, pain at the passive mobilization of the limb. On the other hand other surgeons suggest doing a fasciotomy only in selected group of patients. Therefore, the traumatic rhabdomyolysis has few diagnostically problems. On the other hand, their treatment is complex and must have a multidisciplinary approach. So the rhabdomyolysis actually remain a severe disease with high mortality caused principally by visceral lesions related to sepsis.

Bone mineralization and body composition in young patients with celiac disease.

OBJECTIVES: The aim of the study was to establish whether body composition in patients with celiac disease is normal and influenced by the age at diagnosis or by the duration of the gluten free diet. METHODS: A group of 66 children with celiac disease and 76 healthy controls were studied by dual energy x-ray absorptiometry. We compared celiac patients with the control group, and within the celiac disease group, we compared patients with different age at diagnosis (28 diagnosed in the first 24 months vs 38 later) and with different duration of the diet (16 in gluten free diet for less than 12 months, 11 for less than 24 months, and 39 for more than 24 months). RESULTS: Overall we did not find any significant difference in body composition between overall celiac patients and controls. However the fat mass, the body mass index, and the spine bone mineral density values in late diagnosed celiac patients were significantly lower than in early diagnosed patients (significance values were p < 0.009; p < 0.002; p < 0.002, respectively). Patients on diet for less than 12 months showed significantly lower bone mineral content and density than those on diet for more than 24 months (significance values were, respectively, p < 0.011 and p < 0.022). Spine mineral density was the only parameter significantly influenced both by age at diagnosis (p < 0.03) and duration of gluten free diet (p < 0.008). CONCLUSIONS: Only an early diagnosis of celiac disease in pediatric age and a strict gluten free diet, lasting more than 12 months, allow celiac patients to reach a normal mineralization.

[Television and eating disorders. Study of adolescent eating behavior]. / La televisione e i disturbi del comportamento alimentare. Un'indagine in età adolescenziale.

BACKGROUND: The media, mainly TV, play a significant social and cultural role and may affect the prevalence and incidence of eating disorders such as bulimia and anorexia nervosa. Their influence acts mainly by favoring a tall and thin body as the only fashionable for female adolescents: your social success depends primarily and totally by your physical appearance and you can, (and must), shape your body as you like better. Our research aims t analyze the attitude of adolescent people toward the TV and to investigate on: 1) time spent watching TV programs; 2) the influence of TV on the personal choices of goods to buy; 3) the ideal body images; 4) choice of TV programs. METHODS: Sixty-seven healthy adolescents (36 F-31 M) were included in our study as controls together with 24 female adolescents with eating disorders (DCA) diagnosed according to the DSM-IV and EAT/26 criteria. RESULTS: Our results show a psychological dependence of DCA adolescents from the TV (longer period of time spent watching TV programs, buying attitudes more influenced by TV advertising). The thin and tall body image is preferred by the DCA girls as well as by the controls; however the body appearance and proportions have a predominant and utmost importance only for the eating disorder females. The masculine subjects instead have a preference for a female and masculine opulent body appearance. CONCLUSIONS: To prevent the observed increase in prevalence and incidence of eating disorders among adolescents, it is appropriate to control the messages, myths and false hood propagated by media, TV in particular.