Todo sobre una entidad poco conocida: el síndrome linfoproliferativo postrasplante / All about a little known entity: post transplant lymphoproliferative syndrome

Resumen El síndrome linfoproliferativo postrasplante (SLPT o PTLD por sus siglas en inglés, posttransplantation lymphoproliferative disorder) consiste en un grupo heterogéneo de enfermedades linfoproliferativas que ocurren en el marco de la inmunosupresión postrasplante, que pueden abarcar desde una simple hiperplasia linfoidea hasta un linfoma maligno de alto grado, con eventual evolución fatal. Se estima su desarrollo entre el 1 y el 20% de los pacientes trasplantados. Los principales factores asociados con el desarrollo de SLPT son el grado de inmunosupresión y el virus de Epstein Barr (VEB). La mayoría suceden dentro del primer año postrasplante, pero el riesgo de desarrollarlo continúa hasta los 10 años. Su presentación es variable, puede ser asintomático o con manifestaciones inespecíficas (fiebre, linfadenopatías), lo que dificulta su diagnóstico desde el punto de vista clínico. Por este motivo, los métodos de imagen cumplen un rol fundamental en su diagnóstico, siendo la tomografía computada (TC) el más utilizado. Se deberá sospechar desde las imágenes en todo paciente trasplantado con afección nodal, principalmente en retroperitoneo y mesenterio; y/o extranodal, como el tracto gastrointestinal, órganos y el sistema nervioso central. El objetivo del presente trabajo consiste en realizar una revisión sobre el SLPT mediante las imágenes y conocer la importancia de su sospecha y diagnóstico.

Abstract Post-transplantation lymphoproliferative disease (PTLD) is a heterogeneous group of lymphoproliferative diseases in behave of posttransplant immunosuppression, which ranges from relatively benign lymphoid hyperplasia to poorly differentiated lymphoma, affecting different organs with fatal evolution, eventually. PTLD constitutes a disease with an increasing incidence and detection, estimating its development between 1 and 20% of transplant patients. Although it is based on a multifactorial etiology, the main factors associated with the development of PTLD are the degree of immunosuppression and the Epstein Barr virus (EBV). Most cases of this disorder occur during the first year posttransplant, however, the risk of developing is included in the next 5 to 10 years. The clinical presentation is variable, and the patient may be asymptomatic, or with nonspecific manifestations such as fever, lymphadenopathy, or digestive symptoms, making it difficult to diagnose this entity from clinical suspicion. Imaging plays a fundamental role in diagnosis of PTLD, with the computed tomography (CT) being the most widely used. Findings can mimic those lymphoproliferative processes in patients with no transplant and should be suspect in every transplant patient with nodal affection, such as retroperitoneum and mesenteric¸ or extranodal compromise, like gastrointestinal tract, solid organs, and central nervous system. The aim of this paper is to revise all about the PTLD trough different imaging methods and to know the importance of its suspicion and diagnosis.

Exanguinotransfusión. Experiencia de un hospital de tercer nivel / Exchange transfusion. Experience of a third level hospital

Introducción: Un porcentaje de recién nacidos (RN) a término sanos desarrollan ictericia clínica en la primera semana de vida. El objetivo principal del tratamiento es evitar la aparición de encefalopatía. La tendencia actual al alta precoz en las plantas de maternidad se ha relacionado con un aumento de la incidencia de hiperbilirrubinemia, que requiere tratamiento intensivo. La exanguino transfusión (ET) es una técnica que, aunque invasiva, permite disminuir rápidamente la concentración de bilirrubina y minimizar el riesgo de daño neurológico. Propósito y objetivos: Conocer las características clínicas y epidemiológicas de los RN que requieren ET y las complicaciones derivadas de la técnica. Material y métodos: Estudio retrospectivo de las historias clínicas de los RN 35 semanas y de 28 días de vida que ingresaron en la Unidad de Neonatología del Hospital Universitario «Reina Sofía» de Córdoba en los últimos 6 años y que requirieron la realización de ET. Se analizaron las siguientes variables: edad gestacional media, sexo, etiología de la ictericia, tipo de lactancia, horas de vida en el momento de la técnica, concentración máxima de bilirrubina sérica, y necesidad o no de repetición. Además, se revisaron las complicaciones asociadas a la técnica y la necesidad de su repetición (AU)

Resultados: Se diagnosticaron 16 RN con hiperbilirrubinemia extrema que requirieron ET. La etiología más frecuente fue la isoinmunización ABO (56,25%), seguida de la enfermedad por Rh (37,5%). No se encontraron diferencias en cuanto al sexo. Un 68,75% eran alimentados con lactancia materna exclusiva. El promedio de estancia hospitalaria fue de 7 días. El efecto adverso más frecuente fue la trombocitopenia. En un caso se detectó un aumento del trabajo respiratorio y bradicardia. No se diagnosticó ningún caso de convulsiones, kerníctero, sepsis o parada cardiaca. En un caso se produjo perforación intestinal puntiforme con buena evolución posterior. Las otoemisiones acústicas y la ecografía cerebral fueron normales en todos los casos. En 2 RN con isoinmunización Rh fue necesario repetir la ET. Conclusiones: La mayoría de las reacciones adversas relacionadas con la técnica son alteraciones hematológicas, asintomáticas y tratables (AU)

Introduction: A percentage of term newborns develop jaundice in the first week of life. The main purpose is to avoid the onset of encelopathy. The current tendency of early discharge in maternity hospitals is related to a high incidence of hyperbilirubinemia that requires intensive therapy. Exchange transfusion (ET) is a technique, though invasive allows a rapid decrease of bilirubin and the minimization of the neurological damage risk. Purposes and objectives: To know about the clinical and epidemiological characteristics of the NBs who require exchange transfusion and the complications derived from the technique. Patients and methods: A retrospective study of the medical records of NBs 35 weeks and 28 days of life who were admitted at the Neonatology Unit of Hospital Universitario Reina Sofía de Córdoba (HURS) during the last 6 years and who had to undergo an ET. The following variables have been analyzed: medium gestational age, sex, jaundice etiology, lactation type, hours of life at the moment of the technique, maximum concentration of serum bilirubin, need or not of repetition. The complications associated to the technique and the needs of repetition have also been revised (AU)

Results: 16 newborn with extreme hyperbilirubinemia required exchange transfusion have been diagnosed. The most common etiology was the isoimmunization ABO immunization (56.25% followed by Rh disease (37.5%). There were no differences between sexes. Up to 68.75% of the newborn received exclusive breastfeeding. The mean hospital stay was of 7 days. The most common adverse event was thrombocytopenia. An increase of the respiratory work and bradycardia has been detected in only one case. No case was diagnosed with convulsions, kernicterus, sepsis or heart failure. There was one case with point form intestinal perforation with a later evolution. The acoustic otoemissions and brain echography were normal in all the cases. It was necessary to repeat the ET in 2 NBs with isoimmunization Rh. Conclusions: Most of the adverse events associated with the technique are haematology, asymptomatic and treatable impairments (AU)

¿Es el CRIB un buen predictor de hemorragia intraventricular?. Réplica / Is the CRIB a good predictor of intraventricular hemorrhage?. Reply

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[Utility of the clinical risk index for babies (CRIB) as a predictor of hospital death and intraventricular hemorrhage in very low birth weight and extremely low birth weight neonates]. / Utilidad del CRIB para predecir la muerte hospitalaria y la hemorragia intraventricular en los prematuros de muy bajo peso y extremado bajo peso al nacer.

OBJECTIVE: To assess the utility of the CRIB score as a predictor of hospital death and intraventricular hemorrhage (IVH) in very low birth weight (VLBW) and extremely low birth weight (ELBW) neonates. METHOD: A prospective cohort of VLBW neonates admitted to the neonatal intensive care unit from January 2002 to December 2004 was studied. The data was assessed following the protocol of the SEN 1500 multicenter study. This protocol included assessment of the CRIB score in the first 12 hours of life. Data for the entire group, as well as for two subgroups divided according to birth weight (BW) - VLBW neonates (between 1000 and 1500 g) and ELBW neonates (below 1,000 g) - were evaluated. The area under the receiver operating characteristic curve (Az) was calculated to assess the utility of CRIB score, BW and gestational age (GA). Two multivariate models were used. RESULTS: The cohort consisted of 163 patients. The mean (+/-SD) birthweight was 1.114 (+/-270) g and gestational age (+/-SD) was 29 (+/-3) weeks. The Az for hospital death was 0.757 for the CRIB, 0.758 for BW and 0.703 for GA. The Az for IVH was 0.66 for the CRIB, 0.62 for BW and 0.64 for GA. In the multivariate models for hospital death and IVH, the CRIB was the best predictor. The Az of the CRIB for hospital death was 0.77 for VLBW neonates (p < 0.001) and 0.63 for ELBW neonates (p = 0.82). CONCLUSIONS: The predictive utility of the CRIB for hospital death and IVH is similar to that of BW. In the stratification by groups of weight, we found that the CRIB was the best predictor of hospital death in the group weighing > 1,000 g but was no better than chance in the group weighing < 1,000 g.

Utilidad del CRIB para predecir la muerte hospitalaria y la hemorragia intraventricular en los prematuros de muy bajo peso y extremado bajo peso al nacer / Utility of the clinical risk index for babies (CRIB) as a predictor of hospital death and intraventricular hemorrhage in very low birth weight and extremely low birth weight neonates

Objetivo Evaluar la utilidad del Clinical Risk Index for Babies (CRIB) para predecir la muerte hospitalaria y la hemorragia intraventricular (HIV) en pacientes recién nacidos prematuros de muy bajo peso (RNMBP) y de peso extremadamente bajo al nacer (RNEBP). Método Se siguió de forma prospectiva una cohorte de recién nacidos con peso inferior a 1.500 g ingresados en la unidad de cuidados intensivos neonatales (UCIN), desde enero de 2002 a diciembre de 2004. Se registraron datos de morbimortalidad del estudio multicéntrico SEN-1500, incluida la valoración del CRIB a las 12 h de vida. Se evaluaron los datos globales así como en dos subgrupos según peso: muy bajo peso (entre 1.000 y 1.500 g) y peso extremadamente bajo (< 1.000 g). Se realizaron curvas ROC para estimar el poder de predicción mediante el área bajo la curva (Az) y se procesaron modelos multivariantes. Resultados Se registraron los datos de 163 pacientes. La media de peso al nacer (+DE) fue de 1.114 g (±270) y de edad gestacional (+DE) fue de 29 semanas (±3). El Az del CRIB para la muerte intrahospitalaria fue 0,757, del peso al nacer 0,758 y de la edad gestacional 0,703. El Az del índice CRIB para la HIV fue 0,66, del peso 0,62 y de edad gestacional 0,64. En los dos modelos multivariantes para mortalidad y HIV, el CRIB fue el mejor predictor. En el grupo de RNMBP el Az del CRIB para la muerte hospitalaria fue 0,77 (p < 0,001), siendo 0,63 en el grupo de RNEBP (p = 0,82). Conclusiones La utilidad del CRIB en los recién nacidos menores de 1.500 g para predecir muerte hospitalaria y HIV es similar a la del peso. Al estratificar por grupos de peso, encontramos que en los pacientes mayores de 1.000 g, el CRIB fue el mejor predictor de muerte hospitalaria, mientras que en los menores de 1.000 g el CRIB no fue mejor que el azar

Objective To assess the utility of the CRIB score as a predictor of hospital death and intraventricular hemorrhage (IVH) in very low birth weight (VLBW) and extremely low birth weight (ELBW) neonates. Method A prospective cohort of VLBW neonates admitted to the neonatal intensive care unit from January 2002 to December 2004 was studied. The data was assessed following the protocol of the SEN 1500 multicenter study. This protocol included assessment of the CRIB score in the first 12 hours of life. Data for the entire group, as well as for two subgroups divided according to birth weight (BW) ­ VLBW neonates (between 1000 and 1500 g) and ELBW neonates (below 1,000 g) ­ were evaluated. The area under the receiver operating characteristic curve (Az) was calculated to assess the utility of CRIB score, BW and gestational age (GA). Two multivariate models were used. Results The cohort consisted of 163 patients. The mean (±SD) birthweight was 1.114 (±270) g and gestational age (±SD) was 29 (±3) weeks. The Az for hospital death was 0.757 for the CRIB, 0.758 for BW and 0.703 for GA. The Az for IVH was 0.66 for the CRIB, 0.62 for BW and 0.64 for GA. In the multivariate models for hospital death and IVH, the CRIB was the best predictor. The Az of the CRIB for hospital death was 0.77 for VLBW neonates (p 1,000 g but was no better than chance in the group weighing < 1,000 g

[Prospective study of Candida-related sepsis in the neonate]. / Estudio prospectivo de sepsis por Candida en el recién nacido.

OBJECTIVE: Our objective was to carry-out a prospective study of newborns with systemic candidiasis admitted to our Neonatology Unit in a teritiary hospital during the period of March 1994-September 1997. PATIENTS AND METHODS: To be included in the study the patient had to have Candida sp recovered from a normally sterile body fluid and clinical signs of sepsis. We analyzed perinatal and neonatal antecedents, risk factors, clinical course, diagnosis, treatment and outcome. RESULTS: The incidence of systemic candidiasis was 0.62% (14 newborns). Two were term infants and 12 preterm infants, 9 of which weighed less than 1500 g. All of the patients had as predisposing factors the use of broad spectrum antibiotics, prolonged intravascular catheterization and parenteral nutrition, while 64% had mechanical ventilation. The mean age at onset of sepsis was 22 days, with non-specific clinical presentation. Four infants were treated with intravenous amphotericin B and 9 with liposomal amphotericin B in association with fluconazole in one patient and with flucytosine and fluconazole in another. No adverse effects were observed. Mortality was 21%. C. parapsilosis was isolated in 7 cases and C. albicans in another 7 patients, with an important increase in C. parapsilosis in the last few years. CONCLUSIONS: Clinical suspicion of invasive candidiasis requires the removal of indwelling catheters and early initiation of systemic ungal therapy to reduce mortality. The increased incidence of species with more epidemic presentation like C. parapsilosis reinforce the importance of control measures such as handwashing for all personnel and aseptic management of intravascular catheters and solutions in order to prevent infections.

[Hanhart syndrome (aglossia-adactylia syndrome). Report of 2 cases]. / Síndrome de Hanhart (Síndrome de aglosia-adactilia). A propósito de dos casos.

We discuss two cases of Hanhart syndrome, diagnosed at 3 days and 4 months respectively. Minimal diagnostic criteria (micrognathia and peromelia) are reported, and various etiologic hypothesis are discussed. This syndrome includes others as: aglossia-adactylia, hypoglossia-hypodactylia, oro-acral, oro-mandibular-limb-hipogenesis, ankyloglosia superior, glossopalatine ankylosis, peromelia and micrognathia. Emphasize the impossibility of prevention; the patients may die because of food aspiration, both parents an children being subsidiary of psychological support. The possibilities of logopedical treatment and prothesis of peromelic limb must be evaluated.