Haemophilus influenzae Type b Vaccine Failure in Portugal: A Nationwide Multicenter Pediatric Survey.

Background: Despite the high effectiveness of the Haemophilus influenzae type b (Hib) vaccine in preventing invasive disease (ID) in children, Hib vaccine failures (VFs) cases may still occur. This study aimed to characterize the Hib-VF cases in Portugal in a 12-year period and trying to identify the possible associated risk factors.

Methods: Prospective descriptive nationwide surveillance study.

The Added Value of a Multidisciplinary Clinic for Systemic Autoinflammatory Diseases.

Background: Systemic autoinflammatory diseases (SAID) are characterized by inappropriate activation of the innate immune system and include not only monogenic periodic fever syndromes but also multifactorial conditions. As SAID are rare and represent a diagnostic challenge, a multidisciplinary approach is important to ensure successful diagnosis and adequate follow-up of these patients.

Objective: To describe the organization of our multidisciplinary SAID clinic and to characterize our clinical experience, highlighting the benefits of multidisciplinary team management.

[Correction to the article "Case Series of 103 Children with SARS-CoV-2 Infection in Portugal", published on Acta Med Port 2020 Dec;33(12):795-802].

On page 801, fifth, where it reads: "No início da pandemia, teorizou-se que a vacina BCG pudesse ter um efeito protetor relativamente à COVID-19,27,28 mas não se encontrou até à data evidência para tal, não estando atualmente recomendada a vacinação BCG na prevenção da COVID-19.28,29 No nosso estudo, a maioria dos doentes (76%) tinha sido vacinada. Analisámos separadamente o subgrupo de crianças nascidas após janeiro de 2016, altura em que passaram a ser vacinadas apenas as crianças pertencentes a grupos de risco.

[Case Series of 103 Children with SARS-CoV-2 Infection in Portugal].

Introduction: The North Lisbon University Hospital Center was activated for referral of SARS-CoV-2 infected patients on the 11th March 2020. The aim of this study is to describe the experience at the Department of Pediatrics in the approach and the clinical outcomes of infected children.

Material And Methods: A descriptive observational study was performed.

[Live Vaccine in Children with DiGeorge/22q11.2 Deletion Syndrome].

Introduction: Children with DiGeorge syndrome/chromosome 22q11.2 deletion syndrome might have a variable degree of immunodeficiency, which may limit the use of live vaccines. The aim of this study was to review the adverse effects of live vaccines and possible relation with immune status in patients with DiGeorge Syndrome/partial 22q11.

Chemical characterization of vines grown in incipient volcanic soils of Fogo Island (Cape Verde).

Climate and rich volcanic soils of Fogo Island (Cape Verde) are optimal conditions for grape agriculture. This study aims a first evaluation of the bioaccumulation of essential and non-essential elements in different parts of vines of the same variety (Vitis vinifera L.), grown on recent pyroclasts and lahar deposits.

Propionibacterium Acnes: Cause of Cerebrospinal Fluid Shunt Infection.

Infection after implantation of ventriculo-peritoneal shunts is associated with significant morbidity and mortality. We describe a 9-year-old girl with Propionibacterium acnes shunt infection with negative cerebrospinal fluid cultures, diagnosed by broad-range 16S-rRNA gene polymerase chain reaction. This case supports the use of this molecular diagnostic technique in shunt infections, where the offending pathogens are difficult to culture using traditional methods.

BCG vaccination in patients with severe combined immunodeficiency: complications, risks, and vaccination policies.

Background: Severe combined immunodeficiency (SCID) is a syndrome characterized by profound T-cell deficiency. BCG vaccine is contraindicated in patients with SCID. Because most countries encourage BCG vaccination at birth, a high percentage of patients with SCID are vaccinated before their immune defect is detected.

Congenital malaria in a European country.

A newborn of 26 days, born in Lisbon, Portugal, presented with fever, anaemia and thrombocytopaenia. She was admitted considering neonatal sepsis, but Plasmodium vivax was identified in the second peripheral blood smear performed. Parenteral quinine was started with good evolution.

Human FOXN1-deficiency is associated with αβ double-negative and FoxP3+ T-cell expansions that are distinctly modulated upon thymic transplantation.

Forkhead box N1 (FOXN1) is a transcription factor crucial for thymic epithelium development and prevention of its involution. Investigation of a patient with a rare homozygous FOXN1 mutation (R255X), leading to alopecia universalis and thymus aplasia, unexpectedly revealed non-maternal circulating T-cells, and, strikingly, large numbers of aberrant double-negative αβ T-cells (CD4negCD8neg, DN) and regulatory-like T-cells. These data raise the possibility that a thymic rudiment persisted, allowing T-cell development, albeit with disturbances in positive/negative selection, as suggested by DN and FoxP3+ cell expansions.

[Periodic fever with aphthous stomatitis, pharyngitis and adenitis: report of 21 cases].

Introduction: PFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitis) is a benign sporadic syndrome of unknown cause and an important diagnosis to consider in the child with recurrent fever and tonsillitis.

Objectives: To describe the presentation, age of onset, time until diagnosed, episode duration, frequency of typical and atypical symptoms, clinical course after diagnosis and response to treatment in a case series of children with PFAPA.

Methods: Case series of children with PFAPA referred to our consult over an eight-year period (from May 2001 to May 2009).

First use of thymus transplantation therapy for FOXN1 deficiency (nude/SCID): a report of 2 cases.

FOXN1 deficiency is a primary immunodeficiency characterized by athymia, alopecia totalis, and nail dystrophy. Two infants with FOXN1 deficiency were transplanted with cultured postnatal thymus tissue. Subject 1 presented with disseminated Bacillus Calmette-Guérin infection and oligoclonal T cells with no naive markers.

[Folk medicine using animals in the Chapada Diamantina: incidental medicine?].

This study examines the traditional therapeutic use of animals or parts of animals by an Afro Brazilian population living in the Chapada Diamantina, Bahia, Brazil. The investigation was conducted over a period of eight months and consisted of open- and semi-structured interviews, photographic documentation and identification of species. Besides presenting a list of animal products and their respective use, we seek to stimulate a discussion about the possible existence of a rational basis for the belief in the therapeutic or prophylactic potential of the animal products used in the traditional medicine of Brazilian populations.