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

Depressive and anxiety disorders in chronic hepatitis C patients: reliability and validity of the Patient Health Questionnaire.

UNLABELLED: Depression and anxiety in patients with hepatitis C virus (HCV) infection influence their health-related quality of life and their adherence to antiviral treatment. OBJECTIVE: To study the psychometric properties of the Patient Health Questionnaire (PHQ) as a screening instrument for depression and anxiety in HCV patients. METHODS: Criterion validity, using the structured clinical interview for DSM-IV-Axis I (SCID-I) to diagnose depression and anxiety disorders as diagnostic standard, was evaluated for both summed and algorithm-based PHQ in 500 HCV patients. Correlations with quality of life (Short-Form-36 Health Survey; SF-36) and severity of illness (Hospital Anxiety and Depression Scale; HADS) were calculated to assess construct validity. Test-retest reliability and patients' reaction to the PHQ were assessed in 120 HCV patients. RESULTS: Twenty-three percent of patients (N=114) had a psychiatric disorder (any depressive disorder=18.2%; major depressive disorder=6.4%; generalized anxiety disorder=7.0% and panic disorder=5.8%). The questionnaire demonstrated good test-retest reliability for any depressive disorder (k=.847), major depressive disorder (k=.784), generalized anxiety disorder (k=.787), panic disorder (k=.848), and for any psychiatric disorder (k=.847). There was good agreement between PHQ and SCID-I diagnoses (for any PHQ disorder, k=.821; overall accuracy=90.43%, sensitivity=83.84%; specificity=97.01%). Patients with any PHQ disorder had higher impairment on the SF-36 (p<.001). HADS depression and anxiety scores showed high correlations with PHQ depression (p<.001) and anxiety (p<.001) scores respectively. PHQ administration was well accepted by 97% of patients. CONCLUSIONS: The Spanish version of the PHQ is a reliable, valid, and useful screening instrument to detect depression, generalized anxiety and panic disorders in HCV patients.

Does neuroticism influence cognitive self-assessment after epilepsy surgery?

PURPOSE: To examine how cognitive, personality, and seizure outcome variables influence the subjective cognitive functioning of patients with refractory temporal lobe seizures after epilepsy surgery. METHODS: Thirty-three consecutive patients with drug-resistant partial epilepsy who underwent surgical treatment at a tertiary referral university epilepsy center were tested before surgery and 1 year after surgery. Objective cognitive and subjective cognitive functioning tests were used, and personality was assessed. Seizure control was operationalized as a dichotomous variable. RESULTS: A significant inverse relationship was found between neuroticism and subjective cognitive functioning. None of the other pre- and postoperative cognitive and surgery outcome variables were significant predictors of subjective cognitive functioning, even after controlling for the effect of neuroticism. CONCLUSIONS: Subjective and objective memory functioning are independent in patients with epilepsy after surgical treatment. Subjective memory functioning appears to be related not to seizure relief but to neuroticism. These data suggest that psychological factors such as personality traits predisposing to emotional distress should be taken into consideration in the clinical management and counseling of patients undergoing epilepsy surgery.

Reliability and clinical usefulness of the short-forms of the Wechsler memory scale (revised) in patients with epilepsy.

PURPOSE: The Wechsler Memory Scale-R (WMS-R) is often used for the evaluation of patients with epilepsy, but is time consuming. Two shortened forms of the WMS-R have been published in an effort to reduce the time involved in the test. The purpose of this study is to cross-validate these abbreviated forms in patients with epilepsy. METHODS: We assessed 103 consecutive patients with drug-resistant partial epilepsy being treated in our epilepsy unit. The WMS-R was administered to all patients. The reliability of two shortened versions of the WMS-R were evaluated. The appraisal was performed taking into account the side of the epileptogenic region. The statistical analyses were carried out with the Pearson's correlation and with the intraclass correlation coefficient. RESULTS: Both shortened formulas showed good reliability coefficients for predicting the General Memory and Delayed Recall Indices. In the overall accuracy of the predictions by both short forms no significant differences were found among the three study. groups (right, left or bilateral). However, when considering the predictive error only the three-subtest formulas comprising logical memory, verbal paired associates and visual reproduction efficiently predicted performance, regardless of the side of the epileptogenic region. Of the predicted scores in the overall sample, 92 and 97% fell below the standard error of measurement for general and delayed memory index, respectively. Both short forms correctly classified 89-94% of the performances at the average or impaired level. CONCLUSIONS: The short form of the M-MS-R using three subtests (logical memory, verbal paired associates and visual reproduction) is reliable and time-efficient for estimating the General and Delayed Recall Memory indexes in patients with drug-resistant partial epilepsy.