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Primary immunodeficiencies (PIDs) are rare, undiagnosed and potentially fatal diseases. Clinical manifestations of PID can be fatal or leave sequelae that worsen the quality of life of patients. Traditionally, the treatment of PIDs has been largely supportive, with the exception of bone marrow transplantation and, more recently, gene therapy. The discovering of new affected pathways, the development of new molecules and biologics, and the increasing understanding of the molecular basis of these disorders have created opportunities in PIDs therapy. This document aims to review current knowledge and to provide recommendations about the diagnosis and clinical management of adults and children with PIDs based on the available scientific evidence taking in to account current practice and future challenges. A systematic review was conducted, and evidence levels based on the available literature are given for each recommendation where available., (Copyright © 2020 American Academy of Allergy, Asthma & Immunology and Sociedad Española de Enfermedades Infecciosas y Microbiología Clínica. Publicado por Elsevier España, S.L.U. All rights reserved.)

The aim of our study was to describe the epidemiological features of pediatric patients with diarrhea caused by Aeromonas spp. and to study the antibiotic susceptibility of this agent during a seven-year period. Aeromonas caviae was identified in 93 stool samples from 52.2% males and 85.6% patients younger than 36 months. The season with the lowest number of isolates was winter (14.4%). Co-infection with other diarrheagenic microorganisms was observed in 31.1% of the cases. The largest number of isolates was obtained from Emergency Department samples (45.6%); 43.3% of the patients presented with fever, 87.8% with diarrhea (43% of these cases were associated with pathological products) and 67.8% with vomiting, while 73.3% of the patients did not require hospital admission. Susceptibility higher than 87% was observed to trimethoprim-sulfamethoxazole, ciprofloxacin, cefotaxime and cefepime. All the patients overcame the infectious process and 63.3% of them did not receive any antibiotic treatment during the process. A. caviae was the isolated species associated with intestinal infection. Antibiotic treatment would be specifically indicated in cases selected for their clinical severity., (Copyright © 2019 Asociación Argentina de Microbiología. Publicado por Elsevier España, S.L.U. All rights reserved.)

We present one case of hereditary bisalbuminuria and bisalbuminemia in a Spanish family with three affected members. The double band of albumin was detected accidentally in an routine analytical study of a patient who showed hyperuricemia, this originated the study of the rest of the members of the family. Protein electrophoresis, in serum as well as in urine, showed a double band of albumin, which as in most published cases corresponded to the slow migration type. With immunoelectrophoresis its immune identity with common albumin was established. The biochemical parameters assessed did not show any alteration which could correlate the protein disorder with any associated pathology.