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Non-celiac Gluten Sensitivity in children with headache: a focus on clinical and immunological aspects.
Full text linkINTRODUZIONELe cefalee sono frequenti in età pediatrica. Identificare cause potenzialmente trattabili è obiettivo primario. La cefalea è frequente nelle patologie glutine-correlate, in particolare in Sensibilità al glutine non celiaca (NCGS). La prevalenza di NCGS nei bambini cefalalgici ed i meccanismi etio-patogenetici della cefalea in NCGS non sono chiariti.SCOPI DELLO STUDIOObiettivi dello studio: 1) indagare prevalenza e quadro clinico di NCGS in bambini che presentano anticorpi anti-gliadina nativa (AGA); 2) valutare il profilo di espressione genica in cellule mononucleate del sangue periferico (PBMCs) in pazienti con NCGS; 3) misurare nel siero mediatori solubili identificati mediante gene expression profiling ed anticorpi anti-Tranglutaminasi 6 (TG6), markers di patologie neurologiche glutine-dipendenti.PAZIENTI E METODISono stati considerati soggetti di età <18 anni valutati per cefalea nel periodo 1/03/13 – 31/07/15 presso la Neuropsichiatria Infantile (A..O.U.I. Verona). Criteri di inclusione: 1) cefalea primaria (criteri ICHD III-beta); 2) accettare valutazione di profilo biochimico completo, IgA totali, AGA IgG ed IgA, anticorpi anti-tranglutaminasi 2 IgA (TGA), IgE specifiche per grano, glutine, gliadina. Criteri di esclusione: cefalea secondaria, nevralgia cranica; trattamenti introdotti o modificati negli ultimi 2 mesi; deficit di IgA; soggetti positivi per TGA o IgE specifiche per grano, glutine, gliadina; enteropatia alla biopsia duodenale. Come da protocollo diagnostico per sospetta NCGS, i soggetti AGA-positivi hanno seguito dieta priva di glutine (DPG) per 3 mesi, con successiva reintroduzione (gluten challenge). I soggetti AGA-negativi hanno proseguito la dieta abituale. Per i pazienti NCGS è stata effettuato gene expression profiling in PBMCs. In relazione ai dati di espressione genica, sono stati scelti per dosaggio sierico di CTLA-4 solubile (sCTLA-4) e gp130 solubile (sgp130). E’ stato inoltre effettuato il dosaggio sierico di CD25 solubile (sCD25), marker di attivazione T-linfocitaria, e di anticorpi TG6. RISULTATISono stati reclutati 17 pazienti con emicrania o cefalea muscolo-tensiva: 11 soggetti AGA-positivi (età media 10,6 anni; range d’età 7,9-12,5 anni), 6 soggetti AGA-negativi (età media 11,6 anni; range d’età 9,1-13,8 anni). Nessuno dei soggetti AGA-positivi aveva enteropatia. Sei pazienti AGA-positivi su 11 (55%) hanno ricevuto la diagnosi di NCGS. Sintomi gastrointestinali erano più frequenti in questi pazienti (50% dei soggetti). Le caratteristiche della cefalea ed i sintomi extra-intestinali non sono risultati di supporto per identificare i soggetti NCGS. Tre oggetti, tutti AGA-positivi, presentavano anticorpi anti-TG6; i soggetti con livelli elevati di anticorpi TG6 non hanno avuto miglioramento clinico in DPG per 3 mesi.Il profilo di espressione genica in PBMCs ha documentato up-regolazione di geni correlati ad attivazione di linfociti T e B, geni correlati a linfociti Th17 e geni con signature “Interferone tipo I”.In dieta a normale contenuto di glutine i livelli sierici di sCTLA-4 sono risultati più elevati nei soggetti AGA-positivi. In corso di dieta aglutinata si è osservata riduzione dei livelli di sCTLA4 in tutti i soggetti AGA-positivi, anche se la diagnosi di NCGS non è stata confermata in alcuni. I livelli sierici di sgp130 e sCD25 sono risultati simili nei 3 gruppi di pazienti. CONCLUSIONIUn sottogruppo di pazienti pediatrici con cefalea ha NCGS. I dati di analisi di espressione genica sono suggestivi di una condizione immuno-mediata con signature “Interferone di tipo I”, tipica di malattie autoimmuni. Elevati livelli sierici di sCTLA-4 in soggetti AGA-positivi potrebbero costituire indizio di sensibilizzazione al glutine e coinvolgimento della risposta immunitaria adattativa.Il riscontro di anticorpi anti-TG6 nel siero di pazienti AGA-positivi con cefalea potrebbe essere predittivo di un’insoddisfacente risposta clinica alla dieta aglutinata perseguita per soli 3 mesi.Headaches are frequent in childhood. The identification of any underlying treatable causes is a primary endpoint. Headache is frequent in patients with gluten-related disorders, particularly Non-celiac Gluten Sensitivity (NCGS). Prevalence of NCGS in children with headache and etio-pathogenetic bases for headache in NCGS are unclear.AIMS OF THE STUDYThe study aimed: 1) to explore the prevalence and the clinical picture of NCGS in children with headache and native anti-gliadin antibodies (AGA); 2) to analyze gene expression profiles in peripheral blood mononuclear cells (PBMCs) from children with headache and NCGS; 3) the measurement of soluble mediators in sera of patients identified by means of gene expression profiling, and to determine serum levels of Tranglutaminase 6 (TG6) antibodies, markers of neurological gluten-related disorders.PATIENTS AND METHODSPatients aged <18 years evaluated for headache at the Child Neuropsychiatry Unit, University Hospital of Verona, Italy, in the period 1/03/13 – 31/07/15 were considered for recruitment. Inclusion criteria: 1) primary headache (ICHD III-beta criteria); 2) to accept determination of total IgA, AGA IgG and IgA, anti-tranglutaminase 2 IgA antibodies (TGA), specific IgE to wheat, gluten, gliadin on serum samples. Exclusion criteria: secondary headache, cranial nerve neuralgia; ongoing treatments introduced or modified less than 2 months before; total or partial IgA deficiency; to have tested positive for TGA or IgE to wheat, gluten, gliadin; enteropathy at duodenal biopsy in AGA-positive patients. According to the diagnostic algorithm for NCGS, AGA-positive patients underwent a 3 months – period on gluten-free diet (GFD) followed by reintroduction of dietary gluten for 3 months (gluten challenge). AGA-negative patients continued on their normal diet. The headache clinical course was evaluated by means of standardized scales and questionnaires.PBMCs were collected from NCGS patients in active phase of the disease for gene expression profiling and consequently soluble CTLA-4 (sCTLA-4) and soluble gp130 (sgp130) were chosen to be determined in sera. Soluble CD25 (sCD25), a biomarker for T lymphocyte activation, and TG6 IgA and IgG were also measured in sera. RESULTSSeventeen patients were recruited with migraine or tension-type headache: 11 subjects (mean age, 10.6 years; age range, 7.9-12.5 years) tested positive for AGA, 6 children (mean age, 11.6 years; age range, 9.1-13.8 years) were AGA-negative. None of AGA-positive subjects had enteropathy. Six out 11 AGA-positive patients (55%) received the diagnosis of NCGS. No headache features resulted to be typical of NCGS patients. Gastrointestinal symptoms occurred more frequently in NCGS patients (50%). Extra-intestinal symptoms could not distinguish NCGS patients among other patients.Three patients out 11 (27%) tested positive for TG6 antibodies; all also tested as AGA-positive. Patients with high levels of TG6 antibodies did not show improvement on GFD for 3 months.The gene expression profiling of PBMCs documented up-regulation genes related to T- and B-lymphocyte activation, Th17 cell subset and type I Interferon signature.Levels of sCTLA-4 were higher in AGA-positive patients and markedly reduced on GFD, despite NCGS was not confirmed in some patients. Serum levels of sgp130 and sCD25 were similar in all groups. CONCLUSIONSA subgroup of children with headache has Non-celiac Gluten Sensitivity. Experimental data in NCGS patients suggest the existence of an activated immune response with a type-1 Interferon signature, which is typical of autoimmune diseases. High levels of serum sCTLA-4 in AGA-positive patients may be a clue for gluten sensitization and involvement of adaptive immunity.TG6 antibodies in sera may predict a poor clinical response to GFD upon 3 months – period
Reazioni di ipersensibilità ai vaccini
No full textI vaccini vengono somministrati in ogni fascia d’età e possono causare reazioni avverse, che nella maggior parte dei casi sono note e di lieve entità. Le reazioni allergiche a componenti dei vaccini sono invece rare. Le reazioni di ipersensibilità a vaccini possono essere scatenate dall’antigene immunizzante o da altre sostanze es. adiuvanti, conservanti, residui del processo produttivo. Le manifestazioni dipendono dal meccanismo della reazione e dalla componente responsabile. L'intervallo temporale tra la somministrazione e l'insorgenza dei sintomi è un criterio importante per orientare la diagnosi. Le reazioni allergiche IgE-mediate si presentano nella maggior parte dei casi entro 4 ore dalla somministrazione con orticaria, angioedema, e/o sintomi respiratori o addominali, in varia combinazione. L' anafilassi da vaccino è molto rara. In caso di pregressa reazione allergica al vaccino o sensibilizzazione nota a sostanze in esso contenute è indicata una valutazione specialistica prima di procedere con ulteriori somministrazioni dello stesso vaccino o di sue componenti. Le reazioni di ipersensibilità ritardata si manifestano con dermatite da contatto o noduli in sede di iniezione e sono attribuibili a sostanze diverse dall'agente immunizzante (adiuvanti, conservanti, tracce di antibiotici); di norma non controindicano la vaccinazione se il rapporto rischio/beneficio è a favore della somministrazione. Sono disponibili linee guida per la valutazione e la gestione del paziente allergico ai vaccini, al fine di evitare rischi per il paziente sia per la presenza di controindicazioni, sia in caso di mancata immunizzazione e conseguente esposizione a malattie infettive prevenibili con la vaccinazione
Immune response to Hepatitis B vaccine in patients with celiac disease: A systematic review and meta-analysis
No full textIt is debated whether patients with celiac disease (CD) have non-protective antibody responses to HBV vaccination more frequently than non-affected subjects. To perform a literature review and meta-analysis on protective response to HBV vaccination in CD patients. RCTs and observational controlled studies were eligible. Outcome of interest was an anti-HBs (HBsAb) titer ≥10 IU/L after last vaccine dose. Comparative index was rate ratio (RR). Heterogeneity between studies was addressed and funnel plots were analyzed. Meta-regression models were applied to investigate effect size due to study-specific variables. Twelve retrospective studies on a total of 1,447 participants and 4 prospective studies on 184 subjects were selected. The RR was 0.732 (95% C.I.: 0.664-0.808) and 0.777 (95% C.I.: 0.629-0.960) in the prospective and retrospective studies, respectively. The I(2), indicating heterogeneity, was 51.1% in retrospective, 39.8% in prospective studies. Non-protective antibody responses occurred more frequently in patients than controls. Due to limitations in the available studies, additional trials to evaluate post-vaccination HBsAb titer in CD patients are needed
A postmarket safety comparison of 2 vaccination strategies for measles, mumps, rubella and varicella in Italy
Full text linkIt is strategically important to monitor the safety profile of vaccination schedules in order to achieve and maintain high levels of coverage. We analyzed the cohort of individuals actively invited for measles, mumps, rubella and varicella (MMRV) vaccination in the Veneto region (north-east Italy) from 8/1/2013 to 7/31/2014, assessing the onset of adverse events (AE) relating to 2 different vaccination strategies for MMRV (MMR+V vs MMRV). During the vaccination session at 14 months old, parents were given a form for recording local and systemic reactions to vaccinations for 4 weeks afterwards. Overall, 12,288 forms were returned, and 84.6% of them were included in this analysis (5,130 relating to MMR+V and 5,265 to MMRV); 37.3% of the sample reported no AEs, with no difference between the 2 groups. Local reactions were more common in the MMR+V group (9.6% vs 2.9%; RR 3.33; 95% CI 2.79-3.98), while there was no difference in general reactions between the 2 groups (50% MMR+V vs 52% MMRV). The events most often reported were "fever <39.5°C," which was more frequently associated with the MMRV strategy (p<0.001), and "skin blotches and marks," which occurred more often in the MMR+V group (p<0.001). Reports of "fever ≥39.5°C" were equally distributed between the 2 groups. Sixteen cases of febrile seizures were reported (0.14% in the MMR+V group and 0.17% in the MMRV group). Similar safety profiles were identified for the 2 vaccination strategies. Although the method used to record reactions to vaccination demanded considerable resources, it enabled important information to be collected on parents' perception of the AEs occurring in response to their child's vaccination
Assessing Response Rates and Sleep Disorder Prevalence: Insights from a Propranolol Treatment Study for Infantile Haemangiomas
Full text linkBackground: Infantile haemangiomas (IHs) sometimes require treatment with propranolol. Sleep disturbances are the most frequently reported side effects. Monitoring adverse drug events necessitates repeated hospital visits, which can be challenging during a pandemic.
Objectives: To explore the effectiveness of a new electronic questionnaire in identifying sleep disturbances related to treatment with propranolol and potential confounding factors. To evaluate the response rate to the questionnaire. To report the proportion of patients on propranolol with sleep disturbances.
Methods: In an observational, prospective cohort study, caregivers provided clinical information during ambulatory visits and via an electronic questionnaire after an 8-week treatment course with propranolol and at the time of treatment interruption. Adverse drug reaction reporting forms were assessed for causality.
Results: The questionnaire response rate was 91%, and the completion rate was 100%. A total of 59% of patients experienced sleep disturbances during propranolol treatment, which were considered adverse reactions. Sleep disorders were frequent during sleep regression phases and in subjects who fell asleep during physical contact with caregivers or bed-sharing with parents.
Conclusion: The application of this questionnaire allows for identifying adverse sleep events associated with propranolol in IHs and potential confounders. Counselling on sleep hygiene is recommended before treatment onset
EEG findings during "paroxysmal hemiplegia" in a patient with GLUT1-deficiency
No full textA growing number of studies have disclosed the myriad of features that can suggest the diagnosis of a Glucose-transporter-1 deficiency (GLUT1D). The occurrence of paroxysmal movement disorders such as exercise-induced dystonia and non-kinesigenic dyskinesia, received considerable emphasis, while limited attention has been paid to other paroxysmal phenomena, as transitory neurological disorders. These paroxysmal events are roughly and variably described as limb weakness, hemiparesis or ataxia. Their EEG correlate has been never documented
Hexavalent vaccine and strabismus: data from the Italian spontaneous reporting system
No full textStrabismus is a visual problem in which the eyes are not aligned properly and point in different directions.It is a common condition among children since about 4% of them have strabismus.Single reports of strabismus associated to palsy of cranial nerves attributed to vaccines have been reported2, none of them related to hexavalent vaccine.Strabismus is not reported in the Summary Product Information (SPC) of hexavalent vaccine. Twenty-six cases of strabismus are present in the database, 17 of them related to hexavalent vaccine
Single organ cutaneous vasculitis: Case definition & guidelines for data collection, analysis, and presentation of immunization safety data
No full textVasculitides are a group of heterogeneous conditions character- ized by inflammation of blood vessel wall, which can occur in any organ system. Cutaneous involvement occurs almost exclusively with vasculitis of small and medium-sized vessels [1]. Cutaneous vasculitis (CV) may be: (a) a single organ disease limited to the skin, (b) primary CV with secondary systemic involvement, or (c) a cutaneous manifestation of systemic vasculitis [1]. Several classifications and definitions have been proposed for vasculitides, for example those published by the American College of Rheumatology and the Chapel Hill Consensus Conference (CHCC), but they all have various limitations [2–4]. The prolifera- tion of names for CV is principally due to the fact that various dis- orders can be associated with small-vessel vasculitis of the skin: sometimes it is only cutaneous and in other cases there can be other organ involvement [5]. In 1952, upon the first classification, the term ‘‘hypersensitivity vasculitis” (HV) was coined to distinguish forms of necrotizing arteritis of small vessels from polyarteritis nodosa (PAN), which involved larger vessels. HV derives its name from animal models of vasculitis induced by horse serum or drug administration to cause hypersensitivity reactions [5]. Subsequently, the term has been refined to denote small vessel vasculitis confined mainly to the skin and not associated with any other primary vasculitis (i.e. Henoch-Schoenlein purpura, granulomatosis with polyangiitis or cryoglobulinemia). The 1994 CHCC proposed an alternative term for HV - ‘‘cutaneous leukocytoclastic angiitis” because of the fre- quent failure to identify a precipitant factor for hypersensitivity reaction. This term was also problematic because histological fea- tures were not always consistent with this clinical phenotype [5]. The more comprehensive definition of cutaneous small vessel vas- culitis (CSVV), which includes clinical and histological features of HV and leukocytoclastic vasculitis irrespective of a possible trig- gering factor is also used. More recently, the 2012 revised CHCC nomenclature recommended that for single organ vasculitis, which is applied to vasculitis in arteries or veins of any size in a single organ that has no features that indicate that it is a limited expres- sion of a systemic vasculitis, the involved organ and vessel type should be included in the name (e.g. cutaneous small vessel vas- culitis) [4]. Therefore, for this case definition we adopted the term single organ cutaneous vasculitis (SOCV), which refers to small vessel vasculitis of the skin where systemic involvement has been excluded
Vaccination Management In Children and Adults With Mastocytosis
No full textMastocytosis includes a heterogeneous group of disorders characterized by the presence of clonal mast cells (MC) in cutaneous tissues and extracutaneous organs [1,2]. The disease presents in two primary age-related patterns, which may differ in their clinical manifestations and prognosis [1,2]. Pediatric-onset mastocytosis usually consists of cutaneous disease and tends to resolve itself by adolescence in most cases, in contrast to adult-onset mastocytosis which has a normal chronic course [2]. This article is protected by copyright. All rights reserved
Anaphylactic reactions for vaccines: data from the Italian spontaneous reporting system.
No full textAnaphylactic reactions are rarely reported after vaccination. Aim of this study is to analyze reports of anaphylactic reactions associated to vaccines in the Italian spontaneous reporting database. Case of anaphylactic reactions have been identified through the Standardised MedDRA Query (SMQ) “Anaphylactic reaction” using an algorithmic approach which combines a number of anaphylactic reaction symptoms in order to increase specificity
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