Adverse events related to central venous catheters (CVC) and the influence of CVC characteristics on peripheral blood hematopoietic progenitor cell collection in children.

Introduction: The use of peripheral blood progenitor cells (PBPCs) as a source for hematopoietic stem cell transplantation (HSCT) in pediatric healthy donors is still under debate. The risk of a central venous catheter (CVC) placement and catheter-related complications continue to be the main arguments to discourage its use. Methods: we present a retrospective analysis of 140 PBPC collections in pediatric patients and donors, describing adverse events (AE) related to CVCs as well as the influence of catheterrelated variables on the efficiency of the leukapheresis. Results: 14 CVC-related AEs were recorded (10%). The most common was fever in 5 patients, 4 of which had a catheter-related bacteriemia. Thrombotic events were only observed in 3 patients with active malignancy. A healthy donor presented a moderate bleeding after catheter withdrawal that resolved with local measures, and none of the rest presented any AE. Regarding variables related to the development of AEs, the subject group (patient or donor) was the only one significantly associated (p < 0.0001). Of interest, efficiency was also related to catheter location, being worse in those located in the femoral vein than in into the jugular or the subclavian veins (p < 0.05). In a multivariate analysis, the only variable significantly associated was catheter size (beta 0.238, p < 0.01). Discussion: Placing a CVC for PBPC collection in pediatric subjects is overall safe; CVC-related complications in pediatric healthy donors are very rare. Furthermore, we should try to place catheters of the largest caliber possible, since the efficiency of the collection is related to this variable.

Extracorporeal photopheresis in paediatric patients: A retrospective comparison between different 'off-line' protocols.

BACKGROUND AND OBJECTIVES: Extracorporeal photopheresis (ECP) has been shown to be an effective treatment for graft-versus-host disease (GvHD). However, information regarding lymphocyte collection for ECP in children is limited. The aim of this study was to analyse and compare lymphocyte collection for ECP in children using different devices and protocols. Moreover, we have studied both safety and variables of the infused product related to treatment efficacy. PATIENTS AND METHODS: This was a retrospective study of 91 patients who underwent 1524 apheresis procedures with either the COBE Spectra or Spectra Optia system. The comparison study between the Optia protocols (MNC and CMNC) was prioritized. We analysed 578 procedures using the Optia blood cell separator: 204 and 374 using the MNC and the CMNC protocol, respectively. RESULTS: The Optia CMNC protocol showed better collection efficiency, with increased lymphocyte collection per kg of body weight (p < 0.001). On multivariate analysis, the type of protocol showed no relationship with haematocrit or platelet loss. Most procedures were well-tolerated, with the most frequent adverse events related to venous access (21.7%). Seventy-one percent of patients had either partial or complete clinical GvHD response. In the multivariate model, only two variables were associated with a better response to ECP, younger age and a greater increase of B lymphocytes after treatment. CONCLUSION: Lymphocyte collection for ECP is well-tolerated in most children, achieving complete or partial response in more than half of GvHD patients. CMNC is the optimal software to perform lymphocyte collection in children.

Changes in structural lung disease in cystic fibrosis children over 4 years as evaluated by high-resolution computed tomography.

OBJECTIVES: To compare the worsening of structural lung disease on high-resolution computed tomography (HRCT) with changes in spirometry results in cystic fibrosis (CF) patients, and analyse factors associated with the worsening of structural lung disease over time. METHODS: A total of 31 CF subjects (mean age 11.03 ± 3.67 years old) were prospectively evaluated by two HRCT and spirometry tests performed 4 years apart. HRCT abnormalities were scored using the Bhalla scoring system. Comparisons between changes on HRCT and spirometry were made for all patients, and also for groups categorized by age, sex, genotypic alterations and lung obstruction. RESULTS: The mean HRCT Bhalla scoring, forced expiratory volume in 1 s (FEV1 %pred.) and forced vital capacity (FVC %pred.) were 7.92 ± 3.59, 87.76 ± 20.52 and 96.54 ± 15.12, respectively. There was a significant deterioration in the Bhalla score (p < 0.01) and in certain categories: severity of bronchiectasis, peribronchial thickening, mucous plugging and bronchial divisions. Females had a more pronounced worsening of the Bhalla score than males (p = 0.048). No change over time was found in FEV1 and FVC. Only sex was associated with a deterioration in HRCT. CONCLUSIONS: HRCT Bhalla scoring changes statistically significantly over 4 years, but spirometry results do not. Worsening on HRCT is more evident in females. KEY POINTS: • HRCT Bhalla scoring changes significantly over 4 years, spirometry results do not. • Females experience faster respiratory deterioration than males. • Spirometry results are not associated with deterioration in HRCT.

Pleuropulmonary blastoma as characteristic cause of pneumothorax.

BACKGROUND: Pleuropulmonary blastoma (PPB) is the most common lung neoplasms in childhood. Usually presents as recurrent respiratory infections and in some cases as pneumothorax. CASE REPORT: We report the case of a 2-year-old patient that was diagnosed with PPB, that first manifested as recurrent pneumothorax. Three chest computed tomography were necessary for the diagnosis. The first 2 tomographies showed no abnormalities suggestive of malignancy. The patient had a family history of both PPB and leukemia. Three years and a half after completion of treatment, the patient is in complete remission. CONCLUSIONS: PPB is an uncommon disease but is the most common pulmonary neoplasms in childhood. We must suspect it in patients with a suggestive family history and recurrent pneumothorax in the same location.

Hipoacusia en niños con acueducto vestibular dilatado. Estudio de 55 casos / Enlarged vestibular aqueduct syndrome. A review of 55 paediatric patients

Introducción: El acueducto vestibular dilatado (AVD) es la anomalía congénita más frecuentemente encontrada en técnicas de imagen en hipoacusia neurosensorial infantil. Nuestro objetivo es describir las características clínicas y audiológicas de los niños hipoacúsicos con hallazgo de AVD. Métodos: Estudio retrospectivo de 55 niños diagnosticados de AVD en el periodo 2000–2009. Se analizaron las pruebas audiológicas objetivas y/o subjetivas estándar realizadas según la edad de desarrollo de los niños. Se describen los hallazgos y concomitancias clínicas y audiológicas. Resultados: Treinta y siete pacientes (67,27%) presentaban AVD bilateral y 18 (32,72%) unilateral. La hipoacusia era bilateral en 46 (83,63%) casos y unilateral en 9 (16,36%). La media de edad resultó 3,78 años. Presentaron hipocausa neurosensorial 53 (96,36%) casos (28 bilaterales y profundas), y 2 (3,63%) casos hipoacusia mixta. Tres casos fueron progresivos, 2 fluctuantes, 2 asimétricas y 2 presentaron síntomas vestibulares. Se evidenciaron otras anomalías radiológicas asociadas (6 hipoplasias cocleares, 2 conductos auditivos internos agrandados, 1 vestíbulo dilatado y 1 conducto semicircular horizontal hipoplásico), y 6 síndromes clínicos concomitantes (2 Down, 1 Jacobsen, 1 Pendred, 1 Waardenburg, 1 branquio-oto-renal). Un caso resultó positivo a la mutación GJB2. Se encontró historia familiar de hipoacusia en 12 (21,8%) casos. Conclusión: La presentación clínica de la hipoacusia infantil en el AVD se caracteriza por su variabilidad. Debe incluirse en el diagnóstico diferencial de la hipoacusia mixta. La asociación familiar y sindrómica del AVD deben considerarse en el estudio diagnóstico. Es necesario conocer la historia natural de la enfermedad con fines de información pronóstica a los padres (AU)

Introduction: Enlarged vestibular aqueduct (EVA) is the commonest congenital anomaly found with imaging techniques in paediatric sensorineural hearing loss (SNHL). Our aim was to describe clinical and audiological findings in paediatric hearing loss associated to EVA. Methods: Retrospective review of 55 children with imaging-technique EVA findings from 2000 to 2009. Subjective and/or objective audiological tests were analysed and audiological findings related to clinical features were described. Results: Thirty-seven patients (67.27%) showed bilateral EVA and 18 (32.72%) were unilateral. Hearing loss was bilateral in 46 (83.63%) patients and unilateral in 9 (16.36%). Mean age at diagnosis was 3.78 years. Fifty-three (96.36%) children showed SNHL (28 bilateral and profound), while 2 (3.63%) patients had mixed hearing loss. There were 3 cases of hearing loss progression, 2 fluctuations, 2 of them were asymmetric and 2 patients suffered from vestibular symptoms. Concomitant image findings were 6 cochlear hypoplasia, 2 enlarged internal auditory canals, 1 enlarged vestibule and 1 hypoplastic lateral semicircular canal. Six clinical syndromes were found (2 cases of Down's, and 1 each of Jacobsen, Pendred, Waardenburg and branchio-oto-renal). One child was positive for GJB2 mutation. Familial hearing loss was demonstrated on 12 (21.8%) cases. Conclusion: The clinical picture of hearing loss associated to EVA is characterised by great variability. It should be included in the differential diagnosis of unexplained mixed hearing loss. Familial and syndromic findings have to be taken into consideration in the diagnostic evaluations of such patients. Knowledge about the natural history of this illness is needed so as to give parents prognostic information (AU)

[Enlarged vestibular aqueduct syndrome. A review of 55 paediatric patients]. / Hipoacusia en niños con acueducto vestibular dilatado. Estudio de 55 casos.

INTRODUCTION: Enlarged vestibular aqueduct (EVA) is the commonest congenital anomaly found with imaging techniques in paediatric sensorineural hearing loss (SNHL). Our aim was to describe clinical and audiological findings in paediatric hearing loss associated to EVA. METHODS: Retrospective review of 55 children with imaging-technique EVA findings from 2000 to 2009. Subjective and/or objective audiological tests were analysed and audiological findings related to clinical features were described. RESULTS: Thirty-seven patients (67.27%) showed bilateral EVA and 18 (32.72%) were unilateral. Hearing loss was bilateral in 46 (83.63%) patients and unilateral in 9 (16.36%). Mean age at diagnosis was 3.78 years. Fifty-three (96.36%) children showed SNHL (28 bilateral and profound), while 2 (3.63%) patients had mixed hearing loss. There were 3 cases of hearing loss progression, 2 fluctuations, 2 of them were asymmetric and 2 patients suffered from vestibular symptoms. Concomitant image findings were 6 cochlear hypoplasia, 2 enlarged internal auditory canals, 1 enlarged vestibule and 1 hypoplastic lateral semicircular canal. Six clinical syndromes were found (2 cases of Down's, and 1 each of Jacobsen, Pendred, Waardenburg and branchio-oto-renal). One child was positive for GJB2 mutation. Familial hearing loss was demonstrated on 12 (21.8%) cases. CONCLUSION: The clinical picture of hearing loss associated to EVA is characterised by great variability. It should be included in the differential diagnosis of unexplained mixed hearing loss. Familial and syndromic findings have to be taken into consideration in the diagnostic evaluations of such patients. Knowledge about the natural history of this illness is needed so as to give parents prognostic information.