Prenatally diagnosed segmental intestinal dilatation associated with anorectal malformation.

Introduction: Congenital segmental intestinal dilatation has a low incidence within the differential diagnoses of fetal abdominal cyst masses. Suspicion may arise at prenatal ultrasonography, but diagnosis is confirmed at surgery and subsequently at histopathological analysis. There are various theories available to explain its etiopathogenesis.

Complete ureteropelvic-junction disruption following renal trauma: conservative management.

Introduction: Surgical exploration in complete ureteropelvic-junction disruption (CUPJD) is still recommended by many authors. Conservative approach to pediatric renal trauma (RT) includes minimally invasive techniques such as nephrostomy, angioembolization or double-J stent placement.

Case Report: A 14-year-old patient with CUPJD was treated conservatively.

Haemoglobinopathies and other rare anemias in Spain: ten years of a nationwide registry (REHem-AR).

REHem-AR was created in 2013. The progressive implementation of neonatal screening for haemoglobinopathies in Spanish autonomous communities where the registry had not been implemented, as well as the addition of new centres during this period, has considerably increased the sample of patients covered. In this study, we update our previous publication in this area, after a follow-up of more than 5 years.

Spanish primary care in pediatric trauma (AITP) consensus: An AITP checklist.

Introduction: The course in Primary Care in Pediatric Trauma (ATIP in Spanish) has been taught in Spain since 1997, and there are currently 9 accredited training centers. Care of polytraumatized pediatric patients often takes place in an environment conducive to errors resulting from forgetfulness, which is why checklists - mnemonic tools widely used in industry and medicine - are particularly useful to avoid such errors. Although several checklists exist for pediatric trauma care, none have been developed within the setting of our course.

Trigger-Associated Clinical Implications and Outcomes in Takotsubo Syndrome: Results From the Multicenter GEIST Registry.

Background Takotsubo syndrome is usually triggered by a stressful event. The type of trigger seems to influence the outcome and should therefore be considered separately. Methods and Results Patients included in the GEIST (German-Italian-Spanish Takotsubo) registry were categorized according to physical trigger (PT), emotional trigger (ET), and no trigger (NT) of Takotsubo syndrome.

Thromboelastometry-guided surgery in neuroblastoma complicated with disseminated intravascular coagulation.

Background: Disseminated intravascular coagulation (DIC) is a rare oncological emergency. We report a pediatric neuroblastoma complicated with DIC which required thromboelastometry-guided surgery.

Observation: A 6-year-old female diagnosed with intermediate risk adrenal neuroblastoma developed tumor-related DIC after chemotherapy first cycle.

Antimicrobial stewardship in hospitals: Expert recommendation guidance document for activities in specific populations, syndromes and other aspects (PROA-2) from SEIMC, SEFH, SEMPSPGS, SEMICYUC and SEIP.

In 2012, The Spanish Societies of Infectious Diseases and Clinical Microbiology (SEIMC), Hospital Pharmacy (SEFH), and Preventive Medicine, Public Health and Healthcare Management (SEMPSGS) lead a consensus document including recommendations for the implementation of antimicrobial stewardship (AMS) programs (AMSP; PROA in Spanish) in acute care hospitals in Spain. While these recommendations were critical for the development of these programs in many centres, there is a need for guidance in the development of AMS activities for specific patient populations, syndromes or other specific aspects which were not included in the previous document or have developed significantly since then. The objective of this expert recommendation guidance document is to review the available information about these activities in these patient populations or circumstances, and to provide guidance recommendations about them.

Association Between Preexisting Versus Newly Identified Atrial Fibrillation and Outcomes of Patients With Acute Pulmonary Embolism.

Background Atrial fibrillation (AF) may exist before or occur early in the course of pulmonary embolism (PE). We determined the PE outcomes based on the presence and timing of AF. Methods and Results Using the data from a multicenter PE registry, we identified 3 groups: (1) those with preexisting AF, (2) patients with new AF within 2 days from acute PE (incident AF), and (3) patients without AF.

Update on and future perspectives for the diagnosis of alpha-1 antitrypsin deficiency in Brazil.

Alpha-1 antitrypsin deficiency (AATD) is a rare genetic disorder caused by a mutation in the SERPINA1 gene, which encodes the protease inhibitor alpha-1 antitrypsin (AAT). Severe AATD predisposes individuals to COPD and liver disease. Early diagnosis is essential for implementing preventive measures and limiting the disease burden.

[50 years of the Neonatal Screening Program in Catalonia.].

The Catalonian Newborn Screening Program (CNSP) began in 1969, in Barcelona. It was promoted by Dr. Juan Sabater Tobella and supported by Barcelona Provincial Council and Juan March Foundation.

[Nursing mothers satisfaction with the promotion of breastfeeding and professionals adherence to the recommendations. Multi-center study.].

Objective: The Baby-friendly Hospital Initiative or clinical practice guideline implementation programs have a positive impact on the promotion of breastfeeding (BF). There are knowledge gaps regarding the perceptions of new mothers towards these initiatives, and their degree of satisfaction. Our objective was to describe the satisfaction of BF mothers and adherence to the recommendations of the "Breastfeeding" guide from the Registered Nurses' Association of Ontario (RNAO).

Treatment of balanitis xerotica obliterans in pediatric patients.

Objectives: Balanitis xerotica obliterans (BXO) is a chronic inflammatory disease with a little known incidence in pediatric population. The objective of this work was to describe our experience in the treatment of BXO.

Materials And Methods: Retrospective study carried out in 419 patients undergoing circumcision surgery between January 2014 and January 2017.

Short- and Long-Term Prognosis of Patients With Takotsubo Syndrome Based on Different Triggers: Importance of the Physical Nature.

Background Takotsubo syndrome (TTS) is an acute reversible heart condition initially believed to represent a benign pathology attributable to its self-limiting clinical course; however, little is known about its prognosis based on different triggers. This study compared short- and long-term outcomes between TTS based on different triggers, focusing on various physical triggering events. Methods and Results We analyzed patients with a definitive TTS diagnosis recruited for the Spanish National Registry on TTS (RETAKO [Registry on Takotsubo Syndrome]).

[Pilot experience with indocyanine green navigation in pediatric surgery].

Introduction: Indocyanine Green (ICG) fluorescence is a new tool for navigated minimal invasive and open surgery, with multiple possible uses, that can increase safety and improve surgical results, facilitating intraoperative decision making. We hereby present our pilot series using ICG navigation in different procedures of pediatric surgery.

Material And Methods: As a proof of concept, between May 2017 and March 2019, we have used this technique as a help for decision making in these scenarios: visualization of the biliary tract, vascular and lymphatic structures, neoplastic tissue and other anatomic landmarks.

[Spanish consensus document for the diagnosis, treatment and management of neurogenic bladder.].

The purpose of this document is to establish practical recommendations on neurogenic bladder (NB) management based on scientific evidence and medical and nursing perspective in Spinal Cord Injury (SCI) Units as a first multidisciplinary consensual approach in Spain. METHODS: This paper reports results from the first modified Delphi consensus building exercise on this procedure. A committee of recognised opinion-leaders in rehabilitation and urology with special interest in NB was constituted.

Prosthetic pulmonary valve and conduit endocarditis in congenital heart disease.

Background: Pulmonary valve replacement is one of the most common procedures in patients with congenital heart disease. Little is known about prosthetic valve endocarditis in this population.

Objectives: To review management and outcomes of pediatric and adult patients with a prosthetic pulmonary valve or right ventricle-to-pulmonary artery conduit infective endocarditis.

microRNA-mediated differential expression of TRMU, GTPBP3 and MTO1 in cell models of mitochondrial-DNA diseases.

Mitochondrial diseases due to mutations in the mitochondrial (mt) DNA are heterogeneous in clinical manifestations but usually include OXPHOS dysfunction. Mechanisms by which OXPHOS dysfunction contributes to the disease phenotype invoke, apart from cell energy deficit, maladaptive responses to mitochondria-to-nucleus retrograde signaling. Here we used five different cybrid models of mtDNA diseases to demonstrate that the expression of the nuclear-encoded mt-tRNA modification enzymes TRMU, GTPBP3 and MTO1 varies in response to specific pathological mtDNA mutations, thus altering the modification status of mt-tRNAs.

[Is the integration of nutritional care in the different assistance levels possible? The Catalan experience. Clinical perspective].

Disease-related malnutrition is highly prevalent in pathologies commonly integrating care complexity. Healthcare models for complexity must include malnutrition detection and approaches, since it is a key factor which has great impact on the patient’s evolution and the consumption of healthcare resources. Malnourished patients present higher hospitalization, complication and mortality rates, higher demand of post-discharge social resources and higher hospital readmission frequency.