Children with extended oligoarticular and polyarticular juvenile idiopathic arthritis have alterations in B and T follicular cell subsets in peripheral blood and a cytokine profile sustaining B cell activation.

OBJECTIVES: The main goal of this study was to characterise the frequency and phenotype of B, T follicular helper (Tfh) and T follicular regulatory (Tfr) cells in peripheral blood and the cytokine environment present in circulation in children with extended oligoarticular juvenile idiopathic arthritis (extended oligo JIA) and polyarticular JIA (poly JIA) when compared with healthy controls, children with persistent oligoarticular JIA (persistent oligo JIA) and adult JIA patients. METHODS: Blood samples were collected from 105 JIA patients (children and adults) and 50 age-matched healthy individuals. The frequency and phenotype of B, Tfh and Tfr cells were evaluated by flow cytometry. Serum levels of APRIL, BAFF, IL-1ß, IL-2, IL-4, IL-6, IL-10, IL-17A, IL-21, IL-22, IFN-γ, PD-1, PD-L1, sCD40L, CXCL13 and TNF were measured by multiplex bead-based immunoassay and/or ELISA in all groups included. RESULTS: The frequency of B, Tfh and Tfr cells was similar between JIA patients and controls. Children with extended oligo JIA and poly JIA, but not persistent oligo JIA, had significantly lower frequencies of plasmablasts, regulatory T cells and higher levels of Th17-like Tfh cells in circulation when compared with controls. Furthermore, APRIL, BAFF, IL-6 and IL-17A serum levels were significantly higher in paediatric extended oligo JIA and poly JIA patients when compared with controls. These immunological alterations were not found in adult JIA patients in comparison to controls. CONCLUSIONS: Our results suggest a potential role and/or activation profile of B and Th17-like Tfh cells in the pathogenesis of extended oligo JIA and poly JIA, but not persistent oligo JIA.

Traumatic laryngeal fracture: A case report.

BACKGROUND: The blunt laryngotracheal trauma is a potentially life-threatening infrequent cause of injury that can present with subtle symptoms which often lead to delay in diagnosis if not suspected. CASE PRESENTATION: The authors report a case of a 16-year-old adolescent admitted to the emergency room after a bicycle accident with isolated blunt cervical trauma subsequent to headphones use. The patient presented with odynophagia, hemoptysis, edema, tenderness, linear ecchymosis in the anterior cervical area and no signs of respiratory distress. Cervical computed tomography scan revealed an aligned left side thyroid cartilage fracture. The patient progressed with a complete clinical recovery and at 3 month-follow-up reported no symptoms. CONCLUSIONS: Laryngeal fracture is a rare entity in patients with blunt cervical trauma. A high level of suspicion along with a systematic approach is essential for rapid recognition and early treatment, regardless of the severity of the presenting symptoms, in order to preserve airway and voice functions and improve outcomes.

[ECMO in Neonates with Congenital Diaphragmatic Hernia: The Experience of a Portuguese ECMO Referral Center]. / ECMO em Recém-nascidos com Hérnia Diafragmática Congénita: A Experiência de um Centro de Referência de ECMO em Portugal.

INTRODUCTION: The use of extracorporeal membrane oxygenation (ECMO) is considered by many authors as one of the most important technological advances in the care of newborns with congenital diaphragmatic hernia. The main objective of this study was to report the experience of a Portuguese ECMO center in the treatment of congenital diaphragmatic hernia. MATERIAL AND METHODS: Descriptive retrospective study of newborns with congenital diaphragmatic hernia requiring ECMO support in a Pediatric Intensive Care Unit from January 2012 to December 2019. Data collection using the Extracorporeal Life Support Organization registration and unit data base. RESULTS: Fourteen newborns were included, all with left congenital diaphragmatic hernia, in a total of 15 venoarterial ECMO cycles. The median gestational age was 38 weeks and the median birth weight was 2.950 kg. Surgical repair was performed before entry into ECMO in six, during in seven and after in one newborn. The average age at placement was 3.3 days and the median cycle duration was 16 days. Prior to ECMO, all newborns had severe hypoxemia and acidosis despite optimized ventilatory support, with nitric oxide and inotropic therapy. After 24 hours on ECMO, there was correction of acidosis, improvement of oxygenation and hemodynamic stability. All cycles presented mechanical complications, the most frequent being the presence of clots in the circuit. The most frequent physiological complications were hemorrhagic and embolic (three newborns suffered an ischemic stroke during the cycle). Five newborns (35.7%) died, all associated with complications (two strokes, two massive bleedings and one accidental decannulation). Chronic lung disease, poor weight gain and psychomotor developmental delay were the most frequent long-term morbidities. DISCUSSION: Despite technological advances in respiratory care and improved safety of the ECMO technique, the management of these newborns is complex and there are still several open questions, including the appropriate selection of patients, the best approach and time for surgical correction, and the treatment of pulmonary hypertension in the presence of persistent fetal shunts. CONCLUSION: Survival rate was higher than reported in 2017 Extracorporeal Life Support Organization report (64% versus 50%). Mechanical and hemorrhagic complications were very frequent.

Introdução: A utilização de oxigenação por membrana extracorporal (ECMO) é considerada por muitos autores como um dos maisimportantes avanços tecnológicos nos cuidados de recém-nascidos com hérnia diafragmática congénita. O principal objetivo deste estudo foi reportar a experiência de um centro de oxigenação por membrana extracorporal português no tratamento de hérnia diafragmática congénita.Material e Métodos: Estudo retrospetivo descritivo dos recém-nascidos com hérnia diafragmática congénita com necessidade de suporte de ECMO, numa unidade de Cuidados Intensivos Pediátricos de janeiro de 2012 a dezembro de 2019. Colheita de dados com recurso ao registo da Extracorporeal Life Support Organization e registo da unidade.Resultados: Incluídos 14 recém-nascidos, todos com hérnia diafragmática congénita esquerda, num total de 15 ciclos de ECMO veno-arterial. Mediana de idade gestacional de 38 semanas e de peso ao nascer de 2,950 kg. A correção cirúrgica foi realizada antes da entrada em ECMO em seis, durante em sete e após ciclo em um caso. A mediana de idade de colocação foi de 3,3 dias e a média de duração do ciclo foi de 16 dias. Previamente à ECMO, todos os recém-nascidos apresentavam hipoxemia e acidose grave apesar de suporte ventilatório otimizado, com terapêutica com oxido nítrico e inotrópicos. Após 24 horas em ECMO, verificou-se correção de acidose, melhoria de oxigenação e estado hemodinâmico. Todos os ciclos apresentaram complicações mecânicas, sendo a mais frequente a presença de coágulos no circuito. As complicações fisiológicas mais frequentes foram as hemorrágicas e embólicas (três recém-nascidos sofreram acidente vascular cerebral isquémico durante o ciclo). Cinco crianças (35,7%) morreram, estando todos os casos associados a complicações (duas com acidente vascular cerebral, duas com hemorragia maciça e uma descanulação acidental). A doença pulmonar crónica, má progressão ponderal e atraso do desenvolvimento psicomotor foram as morbilidades a longo prazo mais frequentes.Discussão: Apesar dos avanços tecnológicos nos cuidados respiratórios e melhoria da segurança da técnica ECMO, o manuseamento destes recém-nascidos é complexo e existem ainda várias questões em aberto, incluindo a selecção apropriada dos doentes, amelhor abordagem e tempo de correcção cirúrgica, e o tratamento da hipertensão pulmonar na presença de shunts fetais persistentes.Conclusão: A taxa de sobrevivência foi superior à reportada no relatório da Extracorporeal Life Support Organization de 2017 (64% vs 50%). As complicações mecânicas e hemorrágicas foram muito prevalentes.

Ultrasonographic diagnosis of fetal ovarian cysts: five cases in five years.

OBJECTIVE: To evaluate the outcome of ultrasonographically diagnosed fetal ovarian cysts within a 5-year period. METHODS: This was a retrospective evaluation of the prenatal and postnatal medical records of the five cases of fetal ovarian cyst diagnosed at our institution from January 2002 to December 2006. RESULTS: The mean gestational age at diagnosis was 31.6 weeks. One of the patients had type I diabetes and another had chronic hypertension and preeclampsia. The mean cyst diameter at diagnosis was 38.3 mm (range 29-60 mm). When diagnosed, four of the cysts were simple and the other was septated. During pregnancy, the septated cyst and two of the simple cysts became hemorrhagic. Postnatally the septated cyst and two of the simple cysts underwent spontaneous remission; the other two cases, corresponding to hemorrhagic cysts, were surgically removed after birth. CONCLUSIONS: The best clinical approach to a fetal ovarian cyst is controversial. In this small series, three of the five cysts regressed spontaneously, including a hemorrhagic one. Once a fetal ovarian cyst is diagnosed, ultrasonographic monitoring should be provided in order to identify complications and define the best therapeutic approach.

[Sliding tracheoplasty: a good choice for the treatment of congenital tracheal stenosis]. / Traqueoplastia de deslizamento. Uma boa opção na correcção da estenose congénita da traqueia.

When infants with recurrent wheezing have a clinical course inconsistent with a common diagnosis, anatomic malformations should be considered in diagnostic workup, followed by medical stabilization and early therapy. A careful history and physical examination could reveal important diagnostic clues that indicate the need to perform a fiberoptic bronchoscopy, which in the reported case showed tracheal stenosis in the transition between the first and second third of the trachea. Chest CT scan revealed a long funnel-shaped tracheal stenosis and along with an echocardiogram, excluded associated cardiovascular malformations. Surgical correction by sliding tracheoplasty under extra-corporeal circulation was successfully performed at the Hospital Gregório Marañon in Madrid. The surgical complications that have occurred (mediastinitis and pulmonary embolism) were resolved. After a two-year follow-up, that included endoscopic control, the patient was found to be free of symptoms of wheezing or post-surgical complications. The authors emphasize the need for early diagnosis and surgical treatment to be performed in an experienced reference center.