Inmunodeficiencia Común Variable: manifestaciones clínicas iniciales. Serie de casos / Common Variable Immunodeficiency: initial clinical manifestations. Number of cases

La inmunodeficiencia común variable (IDCV) es una inmunodeficiencia primaria caracterizada por hipogammaglobulinemia de comienzo tardío, que se manifiesta principalmente con infecciones recurrentes Objetivo: describir las manifestaciones clínicas iniciales de pacientes con IDCV diagnosticados en el Hospital de Niños Sor Maria Ludovica entre 1981 y 2019.Diecinueve pacientes fueron incluidos. Todos los pacientes tenían historia de infecciones recurrentes, siendo las más frecuentemente observadas la neumonía (74%) y la otitis media (42%).Se documentó diarrea crónica en 9 pacientes (47%), con malabsorción asociada en 6 de ellos. El 32% de los pacientes presentó desnutrición severa y uno de ellos metaplasia gástrica. Un paciente presentó esplenomegalia y otro síndrome de Evans.Bronquiectasias fueron observadas en el 42% de los pacientes al diagnóstico.Conocer las manifestaciones clínicas iniciales de la IDCV es fundamental para el diagnóstico precoz y tratamiento oportuno

Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by late onset hypogammaglobulinemia, that can manifest as recurrent infections, autoimmunity, digestive disorders and granulomatous disease. Objectives: to describe the initial clinical findings of patients with CVID diagnosed at Hospital de NiñosSorMariaLudovica, between 1981 and 2019. 19 patients were included, 14 were male (74%). All Patients Had a history of recurrent infections, most frequently pneumonia (74%) and acute otitis media (42%). 9 patients suffered from chronic diarrhea (47%), with associated malabsorption in 6 of them. Thirty-two presented with severe malnutrition and 1 patient with gastric metaplasia. One Patient Had Splenomegaly and 1 had Evans´ syndrome. Bronchiectasis were found in 42% of patients at the time of diagnosis. Early suspicion of CVID from pediatricians is essential in order to arrive at a proper diagnosis

Severity of meningococcal infections is related to anthropometrical parameters.

BACKGROUND: Invasive meningococcal infections remain an important cause of death in children. In addition, malnutrition has been classically associated with increased severity of infectious diseases. However, in our experience lethal meningococcaemia in clinically malnourished children is extremely rare. Our purpose was to determine whether there is an association between nutritional status and outcome in children with invasive meningococcal infection. METHODS: We carried out an observational study and prospectively determined anthropometrical parameters in 127 children aged 1 month to 4 years with invasive meningococcal infection seen in our inpatient facilities from August 1999 to May 2004. Severity and survival were the clinical end points analysed. RESULTS: Children with severe disease had higher weight for age (1.02 vs -0.19) and height for age (1.12 vs -0.58) z scores than those with non-severe disease. Non-survivors had higher weight for age (0.90 vs -0.16) and height for age (0.73 vs -0.57) z scores than survivors. Clinical and biological variables usually accepted as predictors of high mortality or severity in patients with meningococcal infection were not significantly associated with weight for age and height for age z scores. CONCLUSION: In the present prospective series of children with invasive meningococcal disease, severity and death were linked to anthropometrical parameters and seemed to be associated with a very good nutritional status, which confirmed our previous uncontrolled observations.