Case report of a familial triple: a syndrome and review of the literature

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The eosinophilic gastroenteritis in children : a report of three cases

La gastroenterite eosinofila (GE) è un raro disordine infiammatorio dell’apparato gastroenterico, caratterizzato dalla presenza di un intenso infiltrato di granulociti eosinofili nella parete di uno o più segmenti gastrointestinali, in assenze di altre cause note di ipereosinofilia. Questa patologia infiammatoria, insieme all’esofagite eosinofila, alla gastrite ed alla colite eosinofila configura il gruppo dei disordini gastrointestinali eosinofili (EGIDs eosinophilic gastrointestinal disorders) che rappresentano l’ eosinofilia primaria del tratto gastroenterico. Descriviamo tre casi clinici relativi a tre bambini di sesso maschile, dell’età alla diagnosi, di 8 e 12 anni, diagnosticati e seguiti nel periodo 2008-2012. La particolarità dei casi descritti risiede nella rara presentazione clinica della gastroenterite eosinofila associata ad ipereosinofilia periferica, in particolare nei due pazienti con coinvolgimento delle sierose, che hanno mostrato, all’esordio, un quadro sintomatologico grave e difficile da inquadrare. La malattia, già di per sé poco frequente in età pediatrica, si è manifestata in un paziente con segni e sintomi di infiltrazione della mucosa intestinale che ha causato episodi di vomito ricorrente associato ad un quadro endoscopico di gastropatia iperemica con lacerazione mucosale a livello dell’esofago terminale, mentre negli altri due casi la presentazione clinica della malattia è avvenuta con un quadro ascitico e grave ipereosinofilia. La presenza di abbondante liquido libero in addome ha permesso, in un caso, l’esecuzione di una paracentesi diagnostica, evidenziando all’esame citologico un tappeto di eosinofili. Relativamente a questa forma (“sierosa-predominante”) di gastroenterite eosinofila ed alla diagnosi citologica avvenuta mediante paracentesi, in letteratura esistono pochi case report di pazienti adulti. La conferma diagnostica di gastroenterite eosinofila è stata affidata, in ogni caso, agli esami endoscopici e ai relativi prelievi bioptici della mucosa intestinale, eseguiti a vari livelli dei segmenti esplorati, che hanno permesso di riscontrare un infiltrato eosinofilo patologico, avendo escluso altre patologie, infettive, autoimmunitarie, neoplastiche che, soprattutto in età pediatrica devono essere sempre considerate nella diagnosi differenziale.The eosinophilic gastroenteritis (EGE) is a rare inflammatory disorder of the gastrointestinal system, characterized by the presence of an intense infiltration of eosinophils in the wall of one or more segments gastrointestinal, in the absence of other known causes of hypereosinophilia. This inflammatory disease, eosinophilic esophagitis, gastritis and colitis eosinophilic to configure the group of eosinophilic gastrointestinal disorders (EGIDs eosinophilic gastrointestinal disorders) that represent the primary eosinophilia of the gastrointestinal tract. We describe three cases related to three male children, age at diagnosis, 8 and 12 years, diagnosed and followed up during the period 2008-2012. The peculiarity of these cases is located in the rare clinical presentation of eosinophilic gastroenteritis associated with peripheral hypereosinophilia, especially in the two patients with involvement of the serosa, which showed, debut, a set of symptoms severe and difficult to define. The disease itself uncommon in children, occurred in a patient with signs and symptoms of infiltration of the intestinal mucosa causing episodes of recurrent vomiting associated with an endoscopic picture of gastropathy with hyperemic mucosal laceration at the level of terminal esophagus, while in the other two cases, the clinical presentation of the disease occurred with ascites and severe hypereosinophilia. The abundant presence of free fluid in the abdomen has allowed, in one case, the execution of a diagnostic paracentesis, highlighting cytological examination a carpet of eosinophils. With regard to this form ("serous-predominant") of eosinophilic gastroenteritis and the cytological diagnosis came through paracentesis, in the literature there are few case reports of adult patients. The diagnostic confirmation of eosinophilic gastroenteritis has been entrusted, in any case, to endoscopic examinations and biopsies to its intestinal mucosa, performed at various levels of the segments explored, which have allowed to find a pathological eosinophilic infiltrate, having excluded other diseases, infectious, autoimmune, neoplastic, especially in children should always be considered in the differential diagnosis

A Giant Congenital Lobular Capillary Haemangioma

Lobular capillary haemangioma is a relatively common benign mucocutaneous lesion in paediatric age. The etiopathogenesis is not very clear, but it seems that lesions originate de novo from a process of lobular vascular proliferation that appears to be neoplastic. Congenital onset is usually very rare; it happens only in 1.1% in literature. We report a case of a newborn with a giant congenital lobular capillary haemangioma of the head (left occipital region) which increased rapidly with superficial ulcers in the early days of life. The baby was operated on by full-thickness excision using the linear closure technique; this treatment allows histological examination, ensures the most efficacy aesthetic results and gives the lowest recurrence rate

Primary Antimicrobial Susceptibility Changes in Children with Helicobacter pylori Infection over 13 Years in Northern Italy

The eradication therapy of Helicobacter pylori (H. pylori) infection is still a challenge for gastroenterologists. One of the main causes of failure in H. pylori eradication is the antibiotic resistance mainly to clarithromycin. Culture from biopsies is maybe the most used method among the antimicrobial susceptibility techniques. In this study, we compared the antimicrobial susceptibility changes in children with H. pylori infection over 13 years and we confirmed that clarithromycin resistance has been increased (16% versus 26%) though with no statistically signficant value. Therefore, clarithromycin should not be used in empiric treatment of H. pylori eradication therapy in children, but its use should be limited only to children with known antimicrobial susceptibility. On the other hand, metronidazole resistance has decreased over this time period in statistically significant manner (56% versus 33%, p=0.014). Furthermore, ampicillin resistance has been confirmed to be very rare (3% versus 0%) in children with H. pylori infection. In conclusion, in H. pylori infection, if we do not know the antibiotic susceptibility of patients, we should recommend an eradication therapy based on the local distribution of antibiotic resistance rates trying to limit the therapeutic failures