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Background Few studies on the age of resolution of Food Protein Induced Enterocolitis Syndrome (FPIES) induced by solid foods are available. In particular, for FPIES induced by egg, the mean age of tolerance acquisition reported in the literature ranges from 42 to 63 months. Objective We have assessed whether the age of tolerance acquisition in acute egg FPIES varies depending on whether the egg is cooked or raw. Methods We conducted a retrospective and multicentric study of children with diagnosis of acute egg FPIES seen in 10 Italian allergy units between July 2003 and October 2017. The collected data regarded sex, presence of other allergic diseases, age of onset of symptoms, kind and severity of symptoms, cooking technique of the ingested egg, outcome of the allergy test, age of tolerance acquisition. Results Sixty-one children with acute egg FPIES were enrolled, 34 (56%) males and 27 (44%) females. Tolerance to cooked egg has been demonstrated by 47/61 (77%) children at a mean age of 30.2 months. For 32 of them, tolerance to raw egg has been demonstrated at a mean age of 43.9 months. No episodes of severe adverse reaction after baked egg ingestion have been recorded. Conclusions It is possible to perform an OFC with baked egg, to verify the possible acquisition of tolerance, at about 30 months of life in children with acute egg FPIES.

Background The study describes the demographic features, culprit foods, clinical features and outcomes for children presenting with acute fish and/or shellfish food protein-induced enterocolitis syndrome (FPIES) in four Italian paediatric allergy centres. Methods A retrospective/prospective study was undertaken. All children diagnosed with fish or shellfish FPIES were enrolled. The diagnosis of FPIES was based on Sicherer's or Miceli Sopo clinical criteria. Skin prick tests (SPT) were performed in all patients, at the time of diagnosis and prior to OFC. Results Seventy children were enrolled. Mean age at first episode was 14 months (range 6–46 months); mean age at diagnosis was 34 months (range 6–164 months). Sole and cod were the fish most commonly implicated. Fifty-seven of 70 (81%) children had FPIES exclusively to fish, 37 of 57 (65%) children had single-fish FPIES, 20 of 57 (35%) multiple-fish FPIES, nine of 70 (13%) presented adverse reactions exclusively to shellfish, and four of 70 (6%) presented adverse reactions to both fish and shellfish. Only four (5.7%) children presented episodes of acute FPIES with different foods (2 to cow's milk, 1 to egg, 1 to beef); in all cases, onset was prior to that of fish or shellfish FPIES. Fifteen of 70 (21%) children tolerated fish other than the offending fish. Twenty-four of 70 (34%) children achieved tolerance (age range 24–102 months). Conclusions The chief peculiarities of acute fish and shellfish FPIES, compared to more frequent cow's milk or soy FPIES, are (i) later age of onset, (ii) longer persistence and (iii) possibility of tolerating fish other than the offending fish. Adverse reactions with shellfish are possible.

Objective To evaluate the utility of a transcranial brain photoplethysmography parameter as a potential marker for patients with multiple sclerosis. Methods We investigated 38 patients affected by multiple sclerosis, according to the revised McDonald criteria (12 males and 26 females, mean age 41.1 ± 8.5 years, Expanded Disability Status Scale mean value 2.6 ± 2.1) and compared them with 20 age- and sex-matched healthy controls. By means of transcranial brain photoplethysmography, a safe and non-invasive technology, we measured the increase in cerebral blood volume during compression for 10 s of the internal jugular veins of both sides in sitting position. Results The cerebral blood volume increase was significantly smaller in the multiple sclerosis patients (left frontal cortex: −58%, p Conclusions Our study reveals that a minor increase in cerebral blood volume on the frontal cortex of both sides in sitting position is associated with a diagnosis of multiple sclerosis and might be a new marker.

A large body of evidence indicates that cerebral hypoperfusion is one of the earliest signs in the development of Alzheimer’s disease (AD). The aim of our study was to evaluate whether the brain reperfusion rehabilitation therapy (BRRT) would improve verbal memory and learning and/or global cognitive impairment in mild AD. Using a prospective, controlled, open-label 12-month study, we enrolled 15 patients with mild AD, who underwent BRRT program (BRRT group), and 10 age–sex-matched mild AD patients, who received no treatment (control group). At baseline (T0), and at the end of the 3 months (T3), 6 months (T6) and 12 months (T12) participants from both groups were given an evaluation, using Mini-Mental State Examination (MMSE) and Rey Auditory Verbal Learning Test (RAVLT). In both groups by using near-infrared spectroscopy, at T0 and T12, we measured tissue oxygen saturation (TOI) on temporal–parietal and frontal cortex of both sides. Ten patients from the BRRT group and 10 from the control group completed the 12-month follow-up. At the end of rehabilitation protocol, a significant improvement of MMSE and RAVLT was observed in the BRRT group as compared to control group. At T12 compared to T0, a significant improvement of TOI on frontal cortex of both sides was observed in the BRRT group as compared to control group. BRRT improves verbal memory–learning and global cognitive impairment which are associated with increased TOI values on frontal cortex of both sides.

The association of adrenocorticotropic hormone (ACTH) insensitivity with achalasia and alacrimia (Allgrove syndrome, 3A) constitutes a rare multisystem disorder. Its evolution is not well known. The aim of this study was to describe clinical and esophageal manometric characteristics and outcomes in Allgrove syndrome.This multicenter retrospective study compared clinical and manometric characteristics at diagnosis and on follow-up of 9 children presenting with 3A (mean age at diagnosis 7.1 years) with those of 9 children with idiopathic achalasia (IA) (mean age at diagnosis 8.3 years).At the time of diagnosis, 3 children with 3A presented with no digestive or respiratory signs because they were identified during a family screening; 1 remained asymptomatic 8 years later. ACTH levels were high in patients with 3A. All of the patients with IA were symptomatic at diagnosis. No significant difference was observed when comparing any of the manometric parameters of the first esophageal manometry of 3A with those of IA. Seven children with 3A were operated on using the Heller procedure, completed by pneumatic esophageal dilation in 2 of these 7. One patient with 3A was treated only by nifedipine. Failure of treatment was observed in 3 children with 3A and 1 child with IA, partial success in 4 with 3A and 1 with IA, and total success in 2 with 3A and 7 with IA (P0.03). Control manometry showed that in the 3A group, partial success after surgery was always associated with abnormally low or normal lower esophageal sphincter (LES) pressure, whereas failure after surgery was associated with high LES pressure.Our data showed that 3A presented a more severe course than IA despite presymptomatic diagnosis in cases of family screening. The high LES pressure noted in some patients with 3A is suggestive of a peculiar pattern in 3A affecting the LES and the lower part of the esophagus.

Background: Faecal calprotectin has been proposed as a sensitive marker for gastrointestinal inflammation in children and adults. High levels have been reported in healthy newborns and during the first months of life; the effect of the kind of feeding on the calprotectin concentration in stools is controversial. Objective: To evaluate faecal calprotectin values in healthy, exclusively breast-fed (BF) or formula-fed (FF) infants. Methods: Stool samples were obtained from 74 healthy infants (39 exclusively BF and 35 exclusively FF) with a median age of 51 days (range 13–90). Exclusion criteria were acute infections and treatment with anti-inflammatory drugs. Stool samples were stored at –20°C until they were analysed, and the faecal calprotectin concentration was detected using a commercial quantitative enzyme-linked immunoassay (Calprest; Eurospital SpA, Trieste, Italy). Results: The median faecal calprotectin concentration was significantly higher in BF infants (555.00 µg/g, range 122.5–2,000.0 µg/g) than in FF ones (206.60 µg/g, range 31.2–797.6 µg/g) (p < 0.001). We observed a significantly higher median stool frequency in BF infants than in FF ones (p < 0.001), but multiple regression analysis (independent variables: kind of feeding and stool frequency; dependent variable: calprotectin) showed a significant coefficient for the kind of feeding, but not for stool frequency (p = 0.937). Conclusions: Our findings show that the kind of feeding influences the faecal calprotectin concentration, with higher values in healthy exclusively BF infants than in FF ones. Our study does not allow us to clearly identify the reason for our finding; this could be due to hormones (such as ghrelin and leptin), cytokines and other immunostimulating and growth factors (such as epidermal growth factor and granulocyte colony-stimulating factor) in human milk, which contribute to the development of the gastrointestinal immune system. Further investigations are needed to better clarify the mechanism underlying the relationship between feeding and faecal calprotectin levels in young infants.

Infliximab (IFX) is efficacious in inducing remission in severe forms of pediatric Crohn's disease (CD). Adult studies indicate that IFX is also safe and well tolerated as maintenance therapy. The present study aimed to evaluate in a prospective manner the efficacy and safety of IFX as maintenance therapy of severe pediatric CD comparing scheduled and "on demand" treatment strategies.Forty children with CD (nonpenetrating, nonstricturing as well as penetrating forms, mean age: 13.9 +/- 2.2 years) with a severe flare-up (Harvey-Bradshaw Index [HBI]or =5, erythrocyte sedimentation rate [ESR]20 mm/h) despite well-conducted immunomodulator therapy (n = 36 azathioprine, n = 1 mercaptopurine, n = 3 methotrexate) combined with steroids were included in this randomized, multicenter, open-label study. Three IFX infusions (5 mg/kg) were administered at week (W)0/W2/W6. At W10, clinical remission (HBI5) and steroid withdrawal were analyzed and IFX responders were randomized to maintenance therapy over 1 year: group A, scheduled every 2 months; group B, "on demand" on relapse.In all, 34/40 children came into remission during IFX induction therapy (HBI: 6.7 +/- 2.5 (WO) vs. 1.1 +/- 1.5 (W10); P0.001). At the end of phase 2, 15/18 (83%) patients were in remission in group A compared to 8/13 (61%) children in group B (P0.01), with a mean HBI of 0.5 versus 3.2 points (group A versus B, P = 0.011). In group A, 3/13 (23.1%) children experienced a relapse compared to 11/12 (92%) children in group B. No severe adverse event occurred during this trial.IFX is well tolerated and safe as maintenance therapy for pediatric CD, with a clear advantage when used on a scheduled 2-month basis compared to an "on demand" basis.

Older age and low socio-economic conditions are associated with poor control of cardiovascular risk factors (RFs). We assessed the prevalence and awareness of cardiovascular RFs in 503 elderly outpatients of low social status attending two public Internal Medicine clinics in Naples, and studied the interaction of education and employment level with risk profile. The therapeutic intervention was oriented to improve patients' motivation through a positive patient-physician relationship, in keeping with the current guidelines for hypertension. The effect of treatment was evaluated by comparing retrospectively the level of cardiovascular RFs at baseline and at the last follow-up examination performed within 31 October 2005. Only 33.3% of patients (age=68+/-6 years) had attended primary school. Overall (current or previous) employment level was also low. Obesity, hypertension and dyslipidaemia were present in most patients, diabetes in 17.3% of them. In all 8.0% of hypertensives, 16.1% of diabetics and 24.7% of dyslipidaemiacs were unaware of their diseases. Cardiovascular risk profile was worse at lower educational and employment levels. Odds ratios for the metabolic syndrome were 0.28 (95% confidence interval (CI)=0.15-0.52) and 0.35 (0.20-0.62) in the most qualified of three education and employment groups, respectively, compared to the lowest ones. The level of all cardiovascular RFs was effectively reduced during treatment. Control rate of most RFs improved significantly (for hypertension, from 12.8 to 36.5%, P

Infant small-bowel intussusceptions, most of the time idiopathic, may exceptionally reveal a severe digestive disease. We report the case of a 4-month-old infant who presented multiple and simultaneous ileal intussusceptions associated with severe acute gastroenteritis. Initially, the infant showed a protein-losing enteropathy with a clear alteration of the general state of health and undocumented fever, resistant to broad-spectrum antibiotic therapy. Skin and splenic nodules associated with hepatosplenomegaly and pancytopenia set in progressively. The etiologic evaluation led to the diagnosis of a Mycobacterium bovis BCG infection related to severe combined immune deficiency. The treatment consisted in anti-tuberculosis quadruple therapy in association with immunoglobin supplementation. Secondarily, the patient underwent gene therapy in a clinical trial. An early BCG vaccine in the first weeks of life, before the outbreak of infection revealing the immune deficiency, is a risk factor in triggering a disseminated BCG infection in immunodepressed infants. This clinical case is the first reported of severe combined immune deficiency revealed by small-bowel intussusceptions related to a disseminated BCG infection.

Background Familial defective apolipoprotein B-100, a dominantly inherited form of hypercholesterolemia caused by a single Arg3500Gln mutation, is silent in childhood but may confer a high risk of cardiovascular disease in adulthood. The objective was to determine the prevalence of familial defective apolipoprotein B-100 in hypercholesterolemic French children and to provide a basis for targeting screening efforts in this population. Methods One hundred ninety children attending 13 pediatric clinics distributed throughout France were included based on the presence of type IIa hypercholesterolemia with a plasma low-density lipoprotein-cholesterol level of more than 130 mg/dL. The Arg3500Gln mutation was detected in dried blood spots using a polymerase chain reaction assay combined with enzymatic restriction. Results Three hyperlipidemia phenotypes were found: monogenic dominant pure hypercholesterolemia (n = 117), polygenic hypercholesterolemia (n = 43), and combined hyperlipidemia (n = 11). Three unrelated children were heterozygous for the Arg3500Gln mutation; all three had monogenic dominant pure hypercholesterolemia (3/94 families; 3.2%), yielding a prevalence of 1.83% (3/164) in hypercholesterolemic children, which is similar to prevalences reported in European adults. Conclusions The familial defective apolipoprotein B-100 mutation was common (1/31) in children with a phenotype of familial hypercholesterolemia, supporting screening in this population with the goal of preventing premature cardiovascular events.

A number of case studies were followed for 10 years. Children suffering from postural defects, structural scoliosis and spinal osteochondrosis were separated from a normal group. Spinal mobility and posture were compared with the mobility of normal, healthy boys and girls aged 5-14 years. In postural defects curves similar to the physiologic one were observed. The range of motion was not physiologic in scoliosis. In this disease, rotation-flexion thoracic hypermobility with decreased lumbar flexion ability was found. A physiologic trend was found in spinal osteochondrosis, although sometimes the rotational mobility of the spine was reduced. An extremely high increase of thoracic kyphosis was found only in children aged 14 years.

Background PEG-based laxatives are considered today the gold standard for the treatment of constipation in children. PEG formulations differ in terms of composition of inactive ingredients which may have an impact on acceptance, compliance and adherence to treatment. We therefore compared the efficacy, tolerability, acceptance and compliance of a new PEG-only formulation compared to a reference PEG-electrolyte (PEG-EL) formulation in resolving faecal impaction and in the treatment of chronic constipation. Methods Children aged 2–16 years with functional chronic constipation for at least 2 months were randomized to receive PEG-only 0.7 g/kg/day in 2 divided doses or 6.9 g PEG-EL 1–4 sachets according to age for 4 weeks. Children with faecal impaction were randomized to receive PEG-only 1.5/g/kg in 2 divided doses until resolution or for 6 days or PEG-EL with an initial dose of 4 sachets and increasing 2 sachets a day until resolution or for 7 days. Results Ninety-six children were randomized into the study. Five patients withdrew consent before starting treatment. Three children discontinued treatment for refusal due to bad taste of the product (1 PEG-only, 2 PEG-EL); 1 (PEG-EL) for an adverse effect (abdominal pain). Intent-to-treat analysis was carried out in 49 children in the PEG-only group and 42 in the PEG-EL group. No significant differences were observed between the two treatment groups at baseline. Adequate relief of constipation in terms of normalized frequency and painless defecation of soft stools was achieved in all patients in both groups. The number of stools/week was 9.2 ± 3.2 (mean ± SD) in the PEG-only group and 7.8 ± 2.4 in the PEG-EL group (p = 0.025); the number of days with stool was 22.4 ± 5.1 in the PEG-only group and 19.6 ± 7.2 in the PEG-EL group (p = 0.034). In the PEG-only group faecaloma resolution was observed in 5 children on the second day and in 2 children on the third day, while in the PEG-EL group it was observed in 2 children on the second day, in 3 children on the third day and in 1 child on the fifth day. Only 2 patients reported mild treatment-related adverse events: 1 child in the PEG-only group had diarrhoea and vomiting and 1 child in the PEG-EL group had abdominal pain requiring treatment discontinuation. The PEG-only preparation was better tolerated as shown by the lower frequency of nausea than in the PEG-EL group. In the PEG-only group, 96% of patients did not demonstrate any difficulties associated with treatment, as compared with 52% of patients in the PEG-EL group (p < 0.001). Also, the PEG-only formulation taste was better than that of PEG-EL (p < 0.001). The difference between the percentage of subjects who took > 80% of the prescribed dose was in favour of the PEG-only group (98% vs. 88%), though it did not reach a conventional statistical level (p = 0.062). Conclusion PEG-only was better tolerated and accepted than PEG-EL in children with chronic constipation. At the higher PEG doses recommended by the manufactures children in the PEG-only group had higher and more regular soft stool frequency than PEG-EL. Trial registration ClinicalTrials.gov: NCT01592734

Objective To evaluate if relative changes in the amplitude of the arterial pulse wave of the cerebral microcirculation (APWCM) measured by near-infrared spectroscopy (NIRS) may provide information about relative changes of cerebral blood flow (CBF) in cerebral cortex. Methods In 10 healthy human volunteers, through simultaneous recording of the APWCM amplitude by means of NIRS and the mean blood flow velocity (MBFV) of middle cerebral artery by means of transcranial Doppler (TCD) at rest and during breath holding and hyperventilation, we evaluate a possible correlation between relative changes of the mean APWCM amplitude and relative changes of MBFV. Results We found a significant linear correlation: breath holding: R 2 0.84, p R 2 0.81, p Conclusion The relative changes of the mean APWCM amplitude seem able to provide information about relative changes of CBF of cerebral cortex in healthy adult humans during breath holding and hyperventilation. Significance APWCM detected by NIRS, a safe, repeatable, inexpensive technology and at the bedside may improve the study of cerebral cortex microcirculation in neurological diseases.

Background and Objective: Tufting enteropathy (TE) is a congenital abnormality of intestinal mucosa development characterized by severe intestinal failure requiring parenteral nutrition (PN) and, in some cases, small bowel transplantation. A few patients have had a more favorable outcome. The objective of this study was to evaluate possible correlations between histological lesion severity in duodenal biopsies and clinical outcomes in children with TE. Patients and Methods: We retrospectively reviewed the records of patients diagnosed with TE between 1993 and 2003 at our institution based on intractable neonatal-onset diarrhea with prolonged dependence on PN and duodenal biopsy findings of villous atrophy, epithelial dysplasia with enterocyte dedifferentiation and disorganization (tufting) of the surface epithelium, and crypt abnormalities. The histological lesions were assessed semiquantitatively and compared with the clinical outcomes including dependence on PN. Results: Seven children, all from consanguineous parents, were studied for a median of 6.5 years. Three were permanently weaned off PN and experienced normal growth without nutritional assistance. Initial biopsies in all 3 children showed severe diffuse histological lesions. At weaning off PN, 2 of these 3 patients had persistent, although less diffuse, histological lesions. Conclusions: Progressive weaning off PN is possible in some children with TE. In our experience, this favorable outcome was not predicted by histological lesion severity, although the lesions improved in some patients. New biomarkers for identifying the histological lesions and predicting the outcome would be useful.

To assess bone status using quantitative ultrasound (QUS) applied to the second metacarpus and to evaluate the influence of vitamin D supplementation on bone mineralization in exclusively breast-fed infants. Seventy seven healthy exclusively breast-fed infants aged

White potato is a very common ingredient in the diet of infants in Mediterranean countries, and in its cooked form, it is one of the first solid foods introduced, usually around the age of 4-6 months. Allergy to potato is uncommon, and allergic reactions to cooked potato have been reported only in children. We report a case of severe potato-induced allergic reaction in an 8-month-old infant with atopic dermatitis and multiple food allergies that raises questions about differential diagnosis between anaphylaxis and food protein-induced enterocolitis syndrome (FPIES).Allergy to cooked potatoes could be a cause of severe although rare allergic reactions; it could be very difficult, in some cases, to make a differential diagnosis between anaphylaxis and FPIES. Moreover, the diagnosis appears to be very important for the choice of therapy and long-term allergologic management.

METHOD:: In this study we sought to characterize the relationship between several pharmacokinetic and pharmacodynamic parameters and virologic responses among HIV/hepatitis C virus genotype-1 co-infected patients receiving pegylated interferon-alpha-2b (peg-IFN2b) and ribavirin. We also tried to establish the underlying mechanisms that lead to poor sustained virologic responder rates observed with African-Americans against Caucasians and compared their results with those observed in a cohort of hepatitis C virus mono-infected patients. RESULTS:: Among our studied population, a viral decline of more than 1.0 log at day 3 combined with viral load of less than 5.0 log IU/ml at day 28 predicted sustained virologic responders with negative predictive value 100% and positive predictive value 100%. African-Americans had significantly (P < 0.01) slower hepatitis C virus viral kinetics as compared to Caucasians. However, peg-IFN2b concentrations and pharmacokinetic parameters, peg-IFN2bmax and peg-IFN2b half-life, were similar in both groups and did not predict sustained virologic responders. Nevertheless, the pharmacodynamic parameter EC50, estimated from nonlinear fitting of the viral kinetics together with peg-IFN2b concentration data, showed that HIV/ hepatitis C virus co-infected African-Americans have lower sensitivity to interferon-alpha thus giving rise to slower viral decline. The combined pharmacokinetic/pharmacodynamic parameter IFNmax/EC90 was an excellent predictor of sustained virologic responders, thus showing the importance of maintaining peg-IFN2b levels above EC90 to achieve successful treatment. CONCLUSION:: Further studies are needed to evaluate whether these pharmacodynamic predictions are a result of differential host response to peg-IFN2b or other viral factors conferring relative resistance to peg-IFN2b.

International audience; BACKGROUND: Childhood-onset Crohn's disease (CD) might reflect a more severe form of disease. To test this hypothesis we analyzed the long-term natural history of CD in an adult cohort of patients with childhood-onset compared to adult-onset CD. METHODS: We selected 206 childhood-onset CD patients among 2992 adult patients with a diagnosis of CD established before December 31, 2000. Disease characteristics were prospectively assessed during follow-up until December 2007 and compared to adult-onset CD patients matched 2 to 1 on gender, year of CD diagnosis, and disease location. RESULTS: Compared to adult-onset CD, patients with childhood-onset CD were more likely to have a severe disease, with an increased year-by-year disease activity index (37% of patient-years in childhood-onset group versus 31% in the adult-onset group, P < 0.001). Immunosuppressant requirement was also increased with a 10-year cumulative risk of 54 +/- 3% in childhood-onset CD group versus 45 +/- 2%, in the adult-onset CD group (P < 0.001). Cumulative risks of stricturing and penetrating complications and surgical resections were not statistically different between groups. Accordingly, these events occurred at a younger age in the childhood-onset CD group. At the age of 30 years the actuarial risk of having undergone an extensive intestinal resection was 48 +/- 5% in the childhood-onset group versus 14 +/- 2% in the adult-onset group (P < 0.001). CONCLUSIONS: Patients with childhood-onset CD exhibit a more active disease and require more immunosuppressive therapy. This feature is observed irrespective of the disease location, suggesting an intrinsic more severe phenotype.

We studied 34 patients with acute ischemic stroke in the territory of the middle cerebral artery (MCA) by three-dimensional transcranial Doppler (TCD-3D). The parameters analyzed were: mean blood flow velocity, systolic and diastolic velocities; indices of pulsatility, hemisphere asymmetry and pulsatility transmission. Of the 34 patients 11 presented marked slowing of flow velocity in the MCA on the infarct side with an asymmetry index (AI) of over 40%, 8 patients with slightly reduced flow velocity in the MCA and an AI of 25-40%, 2 patients in whom there was indirect evidence of collateral circulations in the anterior cerebral artery distribution together with slowing of MCA flow; 5 patients had stenosis of the MCA, 9 patients showed no alterations of the Doppler parameters. The correlation between neurological symptom pattern and AI was significant (r = 0.76). Noninvasive, easy to perform, performable at once and reliable, TCD-3D is a great improvement on traditional transcranial Doppler and is especially useful in assessing the hemodynamics of the cerebral circulation in ischemic stroke.

To study nutritional status in children with chronic bronchitis (CB) in relation with lung function.In this cohort of study, 46 patients aged 6.0 to 17.5 years (mean: 11.9 years) with chronic bronchitis were recruited. None had cystic fibrosis. Body weight, height, skinfold thicknesses, percentage of ideal body weight-for-height (percentage of IBW), body mass index (BMI), BMI Z-score, fat mass and fat-free mass were used to evaluate nutritional status. Arterial blood gases, vital capacity (VC), forced expiratory volume in one s (FEV1), functional residual capacity (FRC) and maximum inspiratory (Pi(max)) and expiratory (Pe(max)) pressures at the mouth were used to evaluate respiratory function.Thirteen children (28%) had malnutrition defined as percentage of IBW lower than 90%, with a predominant fat mass depletion. VC (65+/-13% versus 79+/-15%; p=0.006) and FEV1 (59+/-16% versus 69+/-14%; p=0.03) were significantly lower in children with malnutrition than in children without malnutrition, but no significant differences were observed with regard to the FEV1/VC ratio and blood gases. Pi(max) (56+/-11% versus 88+/-37%, p=0,02) and Pe(max) (46+/-12% versus 58+/-19%, p=0,3) were also lower in children with malnutrition as compared to than without malnutrition.Malnutrition can be observed in children with CB and is associated with significant lower lung function parameters. This could be explained by decrease in respiratory muscle strength.

Achalasia of the cardia is rare in children. We report our experience in the management of 20 children with achalasia.Twenty children (seven girls including two sisters) with achalasia were studied, seven of them had a morbid association. Age at diagnosis ranged from eight months to 18 years (med: 6.4 yrs). Duration of symptoms prior to diagnosis ranged from one to 62 months (med: 8 months). Regurgitations, weight loss and recurrent pneumonias were the most common presenting symptoms. Diagnosis was established using esophageal manometry, which showed aperistalsis throughout the esophageal body with impaired relaxation of lower esophageal sphincter in all children, and chest x-ray and barium esophagram, which were abnormal in 11 and 18 children respectively.Nifedipine used in nine children was ineffective. Two balloon dilatations were performed in one child with a poor result. Surgery with Heller's myotomy combined with an antireflux procedure was performed in 19 children. One child died six months later due to an hypoglycemic coma. During the follow-up (6 months-21 years) symptoms reappeared in five children. Seventy-two percent of the children had no symptoms one year after the surgery and 45%, five years after. Esophageal manometry performed after surgery in seven children showed a normal lower esophageal sphincter pressure but with impaired relaxation, and an aperistalsis throughout the esophageal body. These results justify prolonged follow-up of children with achalasia of the esophagus.

Infliximab, a monoclonal antibody against tumor necrosis factor-alpha, has been shown to be effective for the treatment of refractory Crohn's disease in adult patients, but experience in pediatrics is limited. This retrospective study included 88 children and adolescents, 39 girls and 49 boys, with a median age of 14 years (range 3.3-17.9). Infliximab was indicated for active disease (66%) and/or fistulas (42%) that were refractory to corticosteroids (70%), and/or other immunosuppressive (82%) agents, and/or parenteral nutrition (20%). Patients received 1 to 17 infusions (median 4) of 5 mg/kg (range 3.8-7.3) of infliximab during a median time period of 4 months (1-17 months). Infusion reaction was noted in 13 patients (15%), with a total of 16 reactions in 450 infusions (4%). At Day 90 after the first infusion of infliximab, symptoms improved in 49% of patients, whereas 29% of patients were in remission and 13% of patients relapsed. From Day 0 to Day 90, Harvey-Bradshaw score decreased from 7.5 to 2.8 (P < 0.001), C-reactive protein from 36 to 16 mg/L (P < 0.01), and 1-hour erythrocyte sedimentation rate from 35 to 17 mm (P < 0.01). Dosage of corticosteroids decreased from to 0.59 to 0.17 mg/kg/d (P < 0.001); 53% of patients could be weaned of corticosteroids and 92% of parenteral nutrition. Treatment with infliximab is well tolerated and effective in most children and adolescents with Crohn's disease that is refractory to conventional immunosuppressive therapy. Nevertheless, long-term efficacy remains to be shown, and further studies are urgently needed to precisely determine the best modality of continuing treatment.

The aim of this study was to analyze the population pharmacokinetics of oxcarbazepine (OCBZ) measuring the serum level of its active metabolite, monohydroxylated oxcarbazepine (MHD). We studied a group of patients with symptomatic and cryptogenic epilepsy treated with OCBZ monotherapy orally, at least for 3 weeks. The mean doses, age and weight of the patients were 17.9 +/- 7.8 mg/kg/day, 35.6 +/- 16.4 years and 70.3 +/- 19.2 kg, respectively. Blood samples were taken before the first morning dose of OCBZ and MHD levels were determined by HPLC. A linear relationship was found between OCBZ dose and MHD serum level (r = 0.844, p0.001). The MHD serum concentration (mg/l) can be predicted as 0.85 x OCBZ dose (mg/kg). There was a significant correlation between observed and predicted MHD concentrations for each patient. The mean MHD clearance (Cl/F) calculated was 4.05 +/- 1.69 l/h, with a coefficient variation of 41%. It was independent of dose, age and weight and followed a non normal distribution. The half-life of MHD was 10.50 +/- 3.17 hours. The influence of other antiepileptic drugs on MHD pharmacokinetics was analyzed by comparing the Cl/F medians from groups of patients receiving concomitant drugs with OCBZ monotherapy group where no significant differences were found. The results can be used to estimate a priori OCBZ doses, in order to individualize the treatment.

Authors made a comparison between 167 suffers from Scheuermann's disease (SD), 70 adolescence idiopathic scoliosis (AIS) and 132 age, sex, height, weight, pubertal developmental stages (Tanner stages) matched controls. The height percentile in 130 cases was also determined. The bone mineral density (BMD) was measured by pQCT on the non dominate sided radial bone. The alkaline phosphatase (AP) level increased at the beginning of puberty and in the puberty in SD. In SD the trabecular Z-score of BMD was significantly decreased in Tanner stage 1 to 4 in both boys and girls. It was not found any significance difference, however, in Tanner stage 5, while in AIS girl no significance decrease of BMD was found. In SD good correlation could be demonstrated between increase in AP and decrease in trabecular Z score r = 0.2, while did not correlate with height percentile. The AP level's increase, and radiomorphometric data of SD severity (intervertebral space narrowing and antero-posterior diameter increase of vertebral body) shows a significant correlation with decreased trabecular Z-score in the period of prepuberty.

To compare the relative responsiveness of outcome measures in children with oligoarticular course juvenile chronic arthritis (JCA).Twenty-six consecutive children with JCA andor =4 joints involved were assessed at admission and after 3 months of followup. Outcome measures included physician and parent global assessments, functional ability as measured by the Childhood Health Assessment Questionnaire (CHAQ), articular variables, and laboratory indicators of systemic inflammation. Responsiveness was calculated with the standardized response median, the effect size, and the Guyatt method.The physician global assessment of disease activity, the active joint count, the global articular severity score, and the number and score of swollen joints proved consistently more responsive with either responsiveness measure used. The parent global assessment of the child's well being, the scores of joints with pain/tenderness and limited range of motion, and the number of joints with limited range of motion showed intermediate responsiveness. The CHAQ, morning stiffness, and laboratory indicators of systemic inflammation were the least responsive measures.The subjective estimation of disease activity by the physician and the articular variables were the more responsive outcome measures in children with oligoarticular JCA. The assessment of functional ability as measured by the CHAQ showed a poor sensitivity to clinical change in this subset of patients with JCA.

The purpose of this study was to show a gradient of possible bilateral activation for movements of the non-dominant vs. dominant hand, as well as for areas involved in complex vs. simple hand movements. A standard 1.5 T magnetic resonance imaging (MRI) system has been utilized to localize the cortical motor hand areas, using the blood oxygen level dependent contrast (BOLDc) technique and single-section fast low-angle shot (FLASH) imaging. Ten normal right-handed subjects volunteered for the study. The motor tasks consisted of simple (flexion-extension) finger movements of either hand, and complex movements (finger-to-thumb opposition in a repeating, pre-planned sequence) of the non-dominant hand. Simple movements caused contralateral activation of the primary motor area (MA); ipsilateral activation was observed for the non-dominant hand only. Supplementary motor area (SMA) was also activated, with a clear contralateral prevalence. The ratio of bilateral activation of MA did not change with complex movements of the non-dominant hand, while SMA as well as lateral premotor area were largely bilaterally activated in this task. In conclusion, the ipsilateral MA is activated for movements--even simple--performed with the non-dominant hand. There is widespread functional activity, involving both contralateral and ipsilateral SMA, for complex movements.

The authors review the literature during the past ten years relating to the onset of retroperitoneal hemorrhage during heparin treatment. The phenomenon may be attributed to a thrombotic genesis involving the adrenal glands and may or may not be correlated to the presence of heparin-induced immune phenomena. The severity of the phenomenon is readily understood: given that the pathology is heparin-dependent, suspension of heparin treatment is the first main step to be taken; this is evidently a cause of risk in relation to the pathology that imposes the use of heparin. The rarity of this complication means that, according to the authors, it has been undervalued. They report three cases which were brought to their attention over the past three years. On the basis of their experience, the authors underline the importance of knowing the causes that are supposed to be responsible for the complication and the study of blood coagulative status. In order not to overlook the possible adrenal genesis of the phenomenon they also recommend a careful exploration of the adrenal glands when faced with a retroperitoneal hematoma in which the source of hemorrhage cannot be identified.

Authors analyse their 233 cases of Scheuermann's disease, treated 1980 through 1990 and followed in average until the age of 18.5 years. The material is classified according to the radiological appearance. The assessment of the results was based on the decrease of the dorsal kyphosis, the change of the complaints and of the Radiograms. The efficacity of the braces used in various radiological type. The brace, resting on 3 points, is in atypical form ineffective, in classical appearance success may be expected in 30 per cent from its application. In severe cases, or cases treated only late a brace, resting on 3 points is used for the day and a Milwauke brace in the night. Its efficacity could be increased by the physiotherapeutic ceasing of the flexion contracture of the hip and shoulder and of the ischiocrural tension.

A given population was followed by authors for 10 years. Children suffering from postural defects, structural scoliosis, spinal osteochondrosis were separated from a normal group. Spinal mobility and posture were compared with the mobility of normal, healthy boys and girls aged 5-14 years. In postural defects physiologically curves could be seen. The range of motion hasn't been physiologic in structural scoliosis. The rotation and flexion of the spine were increased while the lumbar flexion was decreased. Physiologic trend was found in Scheuermann disease, however high values of spinal rotation wasn't found. Extremely increased thoracic kyphosis was found only in children aged 14 years. Perhaps the change of motion is an etiologic factor in structural scoliosis.

Local and systemic tumor necrosis factor (TNF) have been shown to have marked tumorcidal effect on VX2 intraocular tumors. Complete ablation or remission of the experimental ocular tumor was not achieved in doses employed. TNF is a potent immune modulator of acute and chronic inflammation. Local toxic effect of TNF to the eye was severe and occurred only in the presence of tumor. Intravitreal TNF will cause severe necrosis of the normal eye. This toxic effect was not seen when intravitreal TNF was injected into a tumor-bearing eye. Chronic perfusion and low doses of TNF in combination with other antitumor agents may be tried in future studies to avoid local and systemic toxicity.

We investigated the levels of circulating CD5+ B cells in 43 patients with seronegative, HLA-B27 negative juvenile arthritis, 7 patients with juvenile dermatomyositis (DM) and 16 children with systemic lupus erythematosus (SLE). We found that CD5+ B cell levels were high in juvenile arthritis (p less than 0.01), normal in juvenile DM and decreased in SLE (p less than 0.01) compared to 33 age matched controls. In juvenile arthritis, the increase of CD5+ B cells appeared to be independent of discuss activity and was present in all the onset types except in a subset of patient with late onset pauciarthritis.

Authors describe their method used in the treatment of camptodactyly. After a 6-year follow-up they report on good functional and esthetic result. In 1 case, even with a late therapy in 5 years of age, considerable improvement could the reached.

No organism from the cradle lives germ-free or allergen-free. The modalities by which infections facilitate conditions of abnormal reactivity, in particular respiratory asthma and allergy to cow milk proteins, are examined. 216 asthmatic children and 50 infants with rotavirus enteritis have been considered. Infections, besides representing stimuli directly projected on the immune system, also constitute factors which more generally influence the way of reacting.

Starting from one case of Gardner's syndrome of their own observation, and in light of Watne's results, the authors argue that in the syndrome just named--and in familial polyposis in a broader sense--genetic factors do not play a predominant role; rather, polyps seem to result from the prolonged action of noxious agents. At any rate, said action can be neutralized, and possibly prevented either with a surgical modification of the distal gut or by treatment with a chemical substance identified as ascorbic acid. In view of these considerations, the authors recommend preventive medical therapy.

The authors, on the basis of their experience on two cases of cardiac tamponade, and from the revision of literature, retain that immediate thoracotomy, followed by re-examination of the pericardial sac and myocardiorrhaphy, represents a treatment preferable to the evacuant pericardiocentesis, confining the latter only to diagnostic conditions and immediate decompression in cases which arrive in unequipped places.

1. A new method has been devised for the nutritional evaluation of food protein quality. The method is analogous to the classical determination of net protein utilization (NPU). The suggested new criterion, the protein utilization by the liver (LPU), expresses the amount of food nitrogen ‘retained’ in the liver as a percentage of the food nitrogen intake.2. Five different foods, casein, soya-bean protein isolate, maize gluten, wheat gluten, cottonseed meal alone or with supplements of amino acids, a total of thirteen samples, were tested for LPU and NPU in groups of six rats. The correlation coefficient between values for LPU and NPU for all seventy-eight rats was +0.85 and was highly significant.

Background Neonatal Lupus Erythematosus (NLE) is an uncommon autoimmune disease characterized by cutaneous, hepatic, hematological, neurological and cardiac involvement. Case presentation Here we report four cases of cutaneous NLE which were referred to our department in the last 10 years and update literature. The newborns presented with different skin, clinical and laboratory features. This underlines the phenotypic variability of NLE. We investigated the passage of maternal antinuclear antibodies (ANA) and extractable nuclear antigen antibodies (ENA) - particularly anti-Ro/SSA, anti-La/SSB and anti-U1 ribonucleoprotein RNP - through the placenta. Despite the positive family background, cutaneous NLE and serological data improved in infants within 4 months without treatment. Conclusion The evolution of cutaneous NLE may be the spontaneous regression of lesions within six months without progression to Systemic Lupus Erytehmatosus.

BACKGROUND AND OBJECTIVE: Older people with inflammatory bowel disease (IBD) appear to have a lower response to anti-tumour necrosis factor (TNF) therapy, with more frequent complications than younger patients. The objective of this study was to assess persistence on therapy and the safety of anti-TNF therapy in older patients (aged ≥ 60 years). METHODS: We retrospectively reviewed the database of the Sicilian Network for Inflammatory Bowel Diseases (SN-IBD), extracting data regarding IBD patients aged ≥ 60 years and controls

We report herein the results of the cross-cultural adaptation and validation into the Italian language of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The Italian CHAQ was already published in the literature and was therefore revalidated while the Italian CHQ was fully cross culturally adapted with 3 forward and 3 backward translations, and than validated. A total of 1,192 subjects were enrolled: 404 patients with JIA (16% systemic onset, 31% polyarticular onset, 21% extended oligoarticular subtype, and 32% persistent oligoarticular subtype) and 788 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA patients, with the systemic, polyarticular and extended oligoarticular subtypes having a higher degree of disability, pain, and a lower overall well-being when compared to their healthy peers. Also the CHQ clinically discriminated between healthy subjects and JIA patients, with the systemic onset, polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the Italian version of the CHAQ-CHQ are reliable, and valid tools for the functional, physical and psychosocial assessment of children with JIA.