Rhinoscleroma in a 5-year-old Portuguese Child

Rhinoscleroma is a chronic granulomatous infectious disease that is rare in Western Europe. We report the case of a 5-year-old Portuguese boy diagnosed with rhinoscleroma in the context of recurrent epistaxis. He had a 6-month course of antibiotic (amoxicillin plus clavulanate) therapy with full recovery.

Anti-N-methyl-D-Aspartate Receptor Encephalitis with Positive Serum Antithyroid Antibodies, IgM Antibodies Against Mycoplasma Pneumoniae and Human Herpesvirus 7 PCR in the CSF

We report the case of a boy with an encephalopathy associated with extrapyramidal and psychiatric symptoms and anti-N-methyl-D-aspartate receptor antibodies. He had positive serum antithyroid antibodies, IgM antibodies against Mycoplasma pneumoniae and human herpesvirus 7 polymerase chain reaction in the cerebrospinal fluid. He was successfully treated with rituximab, after steroids, intravenous immunoglobulin and plasma exchange. The pathophysiology of this disorder may be post-infectious and autoimmune.

Nonlethal, Attenuated, Transfusion-Associated Graft-Versus-Host Disease in an Immunocompromised Child: Case Report and Review of the Literature

Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare complication of transfusion of nonirradiated blood components. It usually affects children in high-risk groups, including those who have primary immunodeficiencies (PIDs). It usually presents with skin, hepatic, digestive, and hematologic involvement and is normally fatal.

Infecções Retrofaríngeas e Laterofaríngeas em Crianças: A Experiência de um Hospital Pediátrico Durante a Última Década

Introdução: Apresentando-se muitas vezes de forma insidiosa e mascarando-se por tratamentos efectuados previamente, as infecções retro e laterofaríngeas são um desafio diagnóstico. São pouco frequentes na era antibiótica moderna, mas têm capacidade para causarem complicações potencialmente fatais. Material e métodos: Estudo retrospectivo dos casos e análise de dados relativos à epidemiologia, etiologia, apresentação clínica, diagnóstico, tratamento e complicações, de crianças diagnosticadas com infecções retro e laterofaríngeas, no nosso hospital pediátrico, desde Janeiro 2001 a Janeiro 2012. Resultados: Foram incluídas no estudo 23 crianças, com idades compreendidas entre os 3 meses e os 8 anos, com uma média de idades de 47 meses (4 anos). Treze (57%) apresentavam infecções retrofaríngeas, 2 (9%) infecções laterofaríngeas e 8 (35%) ambas. A incidência de casos foi maior no ano de 2010 (4 casos). Doze (52%) eram do sexo masculino e 11 (48%) do sexo feminino. A odinofagia (57%), a cervicalgia (26%) e a recusa alimentar (22%) foram as queixas mais comuns à apresentação. A febre (87%), o torcicolo e a rigidez cervical (65%), a tumefacção cervical (52%) e a prostração (35%) foram os achados físicos mais frequentes. Todos (100%) os doentes receberam antibioticoterapia endovenosa. O tratamento médico sem drenagem foi inicialmente proposto para 15 (65%) crianças. A falência no tratamento médico, requerendo cirurgia, ocorreu em 5 (33%) delas. Num dos casos, foi necessário efectuar uma nova drenagem cirúrgica. O tratamento cirúrgico foi inicialmente proposto para 8 (35%) crianças, tendo sido efectuado durante as primeiras 24 horas. Este tratamento não teve falência em nenhum (0%) dos casos, não tendo sido necessária a realização de uma segunda cirurgia. No entanto, numa das crianças, por aparecimento de um novo abcesso noutra localização, houve necessidade de se proceder à sua drenagem. Duas (9%) crianças tiveram complicações: mediastinite, trombose da veia jugular e síndrome de Claude Bernard Horner. Conclusões: Os sintomas na apresentação das infecções retro e laterofaríngeas na população pediátrica são variados, requerendo o seu diagnóstico um elevado índice de suspeição. O tratamento correcto e atempado é fundamental para um prognóstico favorável. O tratamento ideal nos doentes sem obstrução iminente da via aérea é controverso e objecto de debate, particularmente a escolha entre tratamento médico ou cirúrgico como primeira linha. Torna-se portanto, essencial, maior investigação nesta área, de forma a optimizar resultados.

Severe Israeli Spotted Fever with Multiorgan Failure in a Child

An increased risk of severe and fatal Israeli spotted fever (ISF) has been observed in adults, mostly associated with ISF strain. Here, we report a case of severe ISF with multiorgan failure in a Portuguese child.

The Burkholderia Cenocepacia OmpA-Like Protein BCAL2958: Identification, Characterization, and Detection of Anti-BCAL2958 Antibodies in Serum from B. Cepacia Complex-Infected Cystic Fibrosis Patients

Respiratory infections by bacteria of the Burkholderia cepacia complex (Bcc) remain an important cause of morbidity and mortality among cystic fibrosis patients, highlighting the need for novel therapeutic strategies. In the present work we have studied the B. cenocepacia protein BCAL2958, a member of the OmpA-like family of proteins, demonstrated as highly immunogenic in other pathogens and capable of eliciting strong host immune responses. The encoding gene was cloned and the protein, produced as a 6× His-tagged derivative, was used to produce polyclonal antibodies. Bioinformatics analyses led to the identification of sequences encoding proteins with a similarity higher than 96 % to BCAL2958 in all the publicly available Bcc genomes. Furthermore, using the antibody it was experimentally demonstrated that this protein is produced by all the 12 analyzed strains from 7 Bcc species. In addition, results are also presented showing the presence of anti-BCAL2958 antibodies in sera from cystic fibrosis patients with a clinical record of respiratory infection by Bcc, and the ability of the purified protein to in vitro stimulate neutrophils. The widespread production of the protein by Bcc members, together with its ability to stimulate the immune system and the detection of circulating antibodies in patients with a documented record of Bcc infection strongly suggest that the protein is a potential candidate for usage in preventive therapies of infections by Bcc.

The Diet of Preschool Children in the Mediterranean Countries of the European Union: A Systematic Review

This systematic review discusses data on the dietary intake of preschool children living in the Mediterranean countries of the European Union, including the comparison with a Mediterranean-like diet and the association with nutritional status. Specifically, data from the multinational European Identification and Prevention on Dietary and life style induced health effects in children and infants (IDEFICS) study and national studies, such as the Estudo do Padrão Alimentar e de Crescimento Infantil (EPACI) study and Geração XXI cohort in Portugal, ALimentando la SAlud del MAñana (ALSALMA) study in Spain, Étude des Déterminants pré-et postnatals précoces du développement et de la santé de l'ENfant (EDEN) cohort in France, Nutrintake 636 study in Italy, and Growth, Exercise and Nutrition Epidemiological Study in preSchoolers (GENESIS) cohort in Greece, were analyzed. In the majority of countries, young children consumed fruit and vegetables quite frequently, but also consumed sugared beverages and snacks. High energy and high protein intakes mainly from dairy products were found in the majority of countries. The majority of children also consumed excessive sodium intake. Early high prevalence of overweight and obesity was found, and both early consumption of energy-dense foods and overweight seemed to track across toddler and preschool ages. Most children living in the analyzed countries showed low adherence to a Mediterranean-like diet, which in turn was associated with being overweight/obese. Unhealthier diets were associated with lower maternal educational level and parental unemployment. Programs promoting adherence of young children to the traditional Mediterranean diet should be part of a multi-intervention strategy for the prevention and treatment of pediatric overweight and obesity.

Primary Immunodeficiency Associated with Chromosomal Aberration - an ESID Survey

Patients with syndromic features frequently suffer from recurrent respiratory infections, but little is known about the spectrum of immunological abnormalities associated with their underlying chromosomal aberrations outside the well-known examples of Down and DiGeorge syndromes. Therefore, we performed this retrospective, observational survey study.