Premio Dr. Abraham Isaías Kohan - XIII Congreso Argentino de Medicina Transfusional. Proceso de colecta de células progenitoras hematopoyéticas de sangre periférica en pediatría / Dr. Abraham Isaías Kohan Award - XIII Argentine Congress of Transfusion Medicine. Stem cell collections and associated processes in a pediatric population

Fundamento: El trasplante de células progenitoras hematopoyéticas, es una terapéutica utilizada para el tratamiento de pacientes con enfermedades hematológicas y oncológicas, entre otras. Las células progenitoras hematopoyéticas de sangre periférica se obtienen mediante leucaféresis, previa movilización del donante con factores de crecimiento hematopoyético. Objetivos: Comunicar la experiencia de colectas de células progenitoras hematopoyéticas y los procesos asociados, en una población pediátrica candidata a trasplante autólogo o alogeneico. Material y Método: Se evaluaron 53 pacientes y/o donantes para realizar colecta de CPH, entre los años 2008 y 2011. Se tomó consentimiento informado para realizar los procedimientos. Todos fueron evaluados clínicamente y mediante estudios de laboratorio. El momento de colecta se determinó por el número de las células CD34+ en sangre periférica (óptimo 10 a 20 CD34+/uL) en los pacientes y/o donantes, la decisión se tomó en equi­po: médico tratante y de hemoterapia. Resultados: Fueron evaluados 53 candidatos, se realizó colecta en 40: Grupo I autólogo 29 (72,5 %) y Grupo II alogeneico 11 (27,5%). Se realizaron 61 colectas, 50 en Grupo I (82%) y 11 en Grupo II (18%). La mediana de la dosis de movilización con G-CSF fue 12,80 ug/ Kg /día (Rango: 10-25) aplicada entre 4 y 6 días. El recuento de CD34+ en los productos obtenidos resultó en una mediana 6,50 CD34+ x10 6/Kg de receptor (Rango: 1,31-38,34). Conclusiones: Los procesos y procedimientos empleados para obtener células progenitoras hematopoyéticas para el trasplante nos permitieron cumplir los objetivos dentro del programa de garantía de la calidad y obtener resultados clínicos deseados comparables a los publicados en la literatura en este campo.

Background: The hematopoietic stem cell transplantation is a therapy used to treat patients with blood diseases and cancer, among others. Hematopoietic progenitor cells from peripheral blood are obtained by leukapheresis after donor mobilization with hematopoietic growth factors. Objectives: Communicating the experience of stem cell collections and associated processes in a pediatric population candidate for autologous or allogeneic transplantation. Methods: 53 patients and / or donors were evaluated for collection between 2008 and 2011. Informed consent was taken. AII were clinically evaluated and we also performed some laboratory testing. The timing of collection was determined by the number of CD34+ peripheral blood (10 - 20 CD34+ cells /uL) and the decision was made as a team integrated by the physician in charge and the Blood Bank physician.Results: Of the 53 candidates, collection was performed in 40. Group I: autologous 29 (72.5%) and Group II allogeneic 11 (27.5%). 61 collections were made, 50 in Group I (82%) and 11 in Group II (18%). The median dosage of G-CSF mobilization was 12.80 ug/kg/day (range: 10-25) was administered for a period of 4 to 6 days. The CD34+ count in the products resulted in a median of 6,50 x 10 6 CD34+ /kg recipient (range: 1.31 to 38.34). Conclusions: The processes employed in obtaining hematopoietic progenitor cells allowed us to meet goals under the Quality Assurance Program and achieve satisfactory clinical results comparable to those reported in the literature of the field.

Recambio plasmático terapéutico en enfermedades neurológicas pediátricas / Therapeutic plasma exchange in pediatric neurological diseases

El recambio plasmático terapéutico (RPT) es un procedimiento utilizado en el tratamiento de distintas patologías, especialmente las de etiología autoinmune. La base fisiopatológica del RPT consiste en la eliminación de mediadores inflamatorios a través de la extracción de un volumen variable de plasma del paciente y su sustitución por una solución de reposición, usualmente albúmina al 5%; utilizando separadores celulares. Objetivo: analizar la experiencia de nuestra institución en el tratamiento con RPT de pacientes con enfermedades neurológicas. Material y métodos: estudio retrospectivo, descriptivo sobre una población de 43 pacientes con enfermedad neurológica (Miastenia Gravis, Guillain Barré, Enfermedad de Devic, Encefalomielitis diseminada aguda, Polineuropatía desmielinizante inflamatoria crónica y Encefalitis de Rasmussen), tratados con una serie de RPT entre junio 1994 y junio 2009. Resultados: se pudieron evaluar 38 pacientes, por falta de información sobre los 5 restantes, observándose alguna mejoría del cuadro clínico en el 79% de los mismos. En 68% de los RPT se observó una o más complicaciones (hipocalcemia, hipotensión, parestesias). Conclusiones: en nuestra experiencia el recambio plasmático terapéutico constituye un tratamiento efectivo para las enfermedades neurológicas en las que fenómenos autoinmunes juegan un rol importante en la patogénesis, incluso en aquellas con un bajo nivel de evidencia clínica según la categorización de indicaciones de la ASFA.

Therapuetica plasma exchange (TPE) is a procedure used for the treatment of different diseases, especially those of autoimmune etiology. The pathophysiological basis of the TPE is the removal of inflammatory mediators through the extraction of a variable volume of patient plasma and its replacement by a solution, usually albumin 5%, using cell separators. objective: to analyze our institution's experience in the TPE treatment of patients with neurological diseases. Material and methods: a retrospective, descriptive study of a population of 43 patients with neurological disease ( Myasthenia Gravis, Guillain Barre syndrome, Devic's disease, Acute demyelinating polyneuropathy, Rasmussen's encephalitis) treated with a series of TPE between june 1994 and june 2009. Results: 38 patients were able to assess, for lack of information on the remaining 5. We observed some clinical improvement in 79% of them. In 68% of the TPE one or more complications (hypocalcemia, hypotension, paresthesias) were observed. Conclusions: in our experience the therapeutic plasma exchange is an effective treatment for neurological diseases in which autoimmune phenomena play an important role in pathogenesis, even in those with low levels of clinical evidence according to the categorization of indications of the ASFA.

La Escala Colorimetrica para Medición de Hemoglobia: evaluación metodológica de una publicación científicaa sobre pruebas diagnósticas / The colorimetric Scale for Measuring Hemoglibin: systematic evaluatión of a scientific publication about diagnostic test

La Organización Mundial de la Salud ha desarrollado un método colorimétrico para medir la concentración de hemoglobina: Escala Colorimétrica de Hemoglobina (ECH). Esta prueba es de fácil realización, económina y ha sido señalada como potencialmente útil para el diagnóstico de anemia en países en desarrollo. La Escala Colorimétrica de Hemoglobina constituye una herramienta útil para la identificaicón de anemia cuando no se encuentran disponibles hemoglobinómetros digitales u otros méodos de laboratorio. La selección de un método diagnóstico que permita detectar individuos significativametne anémicos en el momento de efectuar la donación de sangre, constituye una etapa fundamental para proteger la salud del donante y del receptor de esa unidad. El método debe ser sencillo, rápido y realizarse preferentemente a partir de una muestra de sangre capilar. El objetivo del presente estuido es evaluar el desempeño de ECH para la selección de donantes de sangre y ejemplificar la metodología que se utiliza para comparar métodos diagnósticos. Realizamos mediciones pareadas de la concentración de hemoglobina con la ECH, y del nivel de hematocrito (n=426), luego evaluamos el desempeño de la ECH comparando los resultados con un método de referencia para la medición de la cocentración de hemoglobina (n=471). Encontramos un elevado número de falsos negativos en la detección de anemia realizada por la ECH, comparando con el microhematocrito y el HemoCue. Las mediciones de Hemoglobina de la ECH mostraron gran variabilidad y poco acuerdo con las del HemoCue. Solo el 62,9% de las mediciones cae dentro de este rango. En conclusión, no recomendamos reemplazar el uso del método de microhematocrito, por la ECH, para la selección de donantes en nuestro banco de sangre.

[Prostatic-vesical inflammation and sexual problems]. / Flogosi prostatovesciocolari e problematiche sessuali.

With the purpose of investigating the relationship between prostatico-vesical inflammation and sexual disturbances, two groups of 15 patients with uncomplicated BPH were treated with mepartricin 150,000 U/die for 60 days. The two groups differed from each other for the presence of sexual disturbances only present in group A. The relationship between BPH and sexual picture was studied on the grounds of both the symptomatic and instrumental variables habitually adopted in the presence of the pathology in question and of a specific method for the quantitative evaluation of dynamic erection. The examination of the results obtained confirmed BPH clinical implications on sexuality as well as mepartricin excellent manageability, confirmed by the fact that never were sexual disturbances brought about by the drug. On the other hand the erectile activity index measured in the two groups, underwent a qualitative improvement even in the absence of changes in the quantitative datum.

[Recent diagnostic and therapeutic aspects in male sexual impotence]. / Recenti aspetti diagnostici e terapeutici dell'impotenza sessuale maschile.

Male sexual impotence is the symptom of an alteration of central and peripheral mechanism neuropsychoendocrine, vascular and neurological. Nowadays it affects 8-10% of sexually active population. In some diseases, like diabetes and uremia, it can reach very high percentages of incidence. At our Andrology Center 35% of referrals are represented by sexual complaints. In the last years the diagnostic accuracy has increased, narrowing the percentage of unknown causes. Vasculopathy represents the most relevant pathological condition associated with impotence: it can affect both arterial and venous vessels. The new medical technologies and procedures permit an increase of the life span but often affecting the quality of life. Therefore, the iatrogenic causes of impotence, both pharmacological and surgical, are growing. A modern diagnostic approach starts with an accurate clinical history and physical examination, followed by an NPT (nocturnal penile tumescence) test and/or ICI (intracavernosal injection) with a standard dose of PGE1 and Doppler flowmetry of penile arteries. An endocrine evaluation (LH, testosterone and prolactin) is also performed. Further investigation of a vascular dysfunction is represented by more invasive procedures, like arteriography, cavernosography and cavernosometry. A suspect of neurological disease is confirmed by sacral evoked potentials. According to the findings of these examinations, a correct therapeutical approach can be applied in 100% of cases. An endocrine treatment is adequate only when a clear reduction of T plasma level or hyperprolactinemia are present. The treatment of other central disorders causing psychoneuroendocrine impotence is promising, but still under investigation. The intracavernosal injection of vasoactive drugs, apart from having revolutionized the diagnostic approach to the impotent patient, represents a clear standpoint in medical management of impotence, particularly in vascular and neurological diseases. The great advancement in the technology of penile prostheses has allowed the development of valuable and reliable tools to be used in selected cases.

[Transdermal therapy of erectile insufficiency]. / La terapia transdermica dell'insufficienza erettile.

The introduction of intracavernosal (i.c.) administration of vasoactive drugs has revolutionized diagnosis and treatment of sexual impotence. This procedure, though, carries some risks and undesired side-effects, such as fibrosis, priapism, hematomas, etc. Thus, at our Centre has been evaluated the possibility of a transdermal (TS), either active or passive, treatment of impotence. In a first trial, double blind crossover, 62 patients have been treated with yohimbine (YOH) as ointment. About 5 mg of the drug were applied at the balanopreputial sulcus, twice daily. In 10 patients YOH was also assayed by HPLC in the blood drawn from the corpora cavernosa after the application of the drug: a rapid adsorption of the drug was demonstrated and a peak value of 58 ng/ml at 25 min. Treatment with YOH was particularly satisfactory in patients with impotence of recent onset and mild degree, that is without major vascular alterations. The active TD drug administration was tested with papaverine delivered with cavernous bodies by a C.T.D.A.S. (Controlled Transdermal Drugs Administration System). Thin layer chromatography has shown the passage of about 10% of patients a relevant amelioration of erectile function was observed.

[The Rigiscan system in the diagnosis of male sexual impotence]. / Il Rigiscan system nella diagnostica dell'impotenza sessuale maschile.

The article reviews historical basis of Nocturnal Penile Tumescence (NPT) test and the evolution of techniques to perform it. From complex and time-consuming instruments, modern technology has brought us to the use of the Rigiscan system. This is a transportable (home or office usable), computerized method to detect change of penile rigidity and circumference during sleep-related spontaneous-or daytime induced-erections. While the instrument was mainly developed to perform NPT tests, it is nowadays used to record penile rigidity in realtime after the intracavernous injection of vasoactive drugs. In our experience, normal parameters to refer for a NPT evaluation are: number of erectile episodes: 4-5 per night; mean duration od episodes: > 30 minutes; increase of penile circumference: > 3 cm (base loop) and > 2 cm (tip loop); maximal rigidity: > 70% (both base and tip). Reference parameters for realtime monitoring after intracavernous injection of PGEI are: latency of response: < 12 minutes; duration (with a stable plateau): > 30 minutes; constant plateau: > 70%; increase of penile circumference: > 3 cm (base) and > 2 cm (tip). The use of these reference parameters, together with the data coming from other procedures (e.g. penile arterial flow with the Doppler), allows a better diagnostic accuracy in the approach to sexual impotence.