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1.
Int. j. morphol ; 38(6): 1618-1622, Dec. 2020. tab, graf
Article in English | LILACS | ID: biblio-1134488

ABSTRACT

SUMMARY: The use of hematological counts for the prevention, diagnosis and follow-up of hematological diseases has increased. Indeed, the correct operation of a clinical laboratory is essential to producing comparable results. However, there is a paucity of validation and reproducibility studies among the different existing methods for clinical analysis. Therefore, our aim was to assess the commutability of the results provided by analyzers with different measuring systems. Sixty venous blood samples were obtained from patients, without discriminating for age or sex. Then, an automated hematological analysis was performed using the Cell-Dyn Ruby and HumaCount 5L instruments. The variables measured were: RBC, Hb, HCT, MCV, MCH and MCHC. The data were compared by a one-way ANOVA and Pearson's correlation coefficient. Statistical significance was fixed at p < 0.05. There were no statistically significant differences for RBC, HCT, MCH or MCHC. In addition, with the exception of MCHC, all the analytes showed a good correlation coefficient between the two instruments. There is a variety of automated systems for the clinical laboratory and it is essential for the clinician to know the different methodologies used in hematological analyzers as well as their sensitivity and specificity. Therefore, our results are useful for demonstrating the importance of practical knowledge of the analyzers mentioned.


RESUMEN: El uso de recuentos de células sanguíneas para la prevención, diagnóstico y monitoreo de enfermedades hematológicas ha ido en aumento. Por ello, el funcionamiento correcto de un laboratorio clínico es indispensable para producir resultados comparables. Sin embargo, existen pocos estudios de validación y reproducibilidad de los diferentes métodos de análisis clínico existentes. Por lo tanto, nuestro objetivo fue evaluar la intercambiabilidad de los resultados entregados por los analizadores que utilizan diferentes sistemas de medición. Se obtuvieron sesenta muestras de sangre venosa de pacientes, sin discriminar por edad o sexo. Los eritrogramas fueron obtenidos utilizando los analizadores automatizados Cell-Dyn Ruby y HumaCount 5L. Las variables medidas fueron: RBC, Hb, HCT, MCV, MCH y MCHC. Los datos fueron comparados por ANOVA a una vía y la correlación de Pearson. La significación estadística se estableció en el nivel estándar p<0,05. No hubo diferencias estadísticamente sig- nificativas para RBC, HCT, MCH y MCHC. Con la excepción de la MCHC, todos los analitos presentaron un buen coeficiente de correlación entre los dos analizadores comparados. Existen varios sistemas de automatización para su uso en laboratorios clínicos. Por lo tanto, es primordial para el clínico estar familiarizado con las diferentes metodologías utilizadas en los analizadores de sangre, así como su sensibilidad y especificidad. Nuestros resultados son útiles para mostrar la importancia del conocimiento práctico de los diferentes sistemas de medidas comparados.


Subject(s)
Humans , Hematologic Diseases/diagnosis , Hematologic Tests/methods , Blood Cell Count/methods , Blood Cells , Hemoglobins , Reproducibility of Results , Analysis of Variance , Sensitivity and Specificity , Erythrocyte Indices , Flow Cytometry , Hematocrit
2.
Rev. cuba. hematol. inmunol. hemoter ; 36(4): e1285, oct.-dic. 2020. tab
Article in Spanish | LILACS, CUMED | ID: biblio-1289413

ABSTRACT

Introducción: La neutropenia febril incide frecuentemente en pacientes con hemopatías malignas y representa una urgencia médica; actualmente su manejo terapéutico constituye un verdadero reto debido a la aparición de infecciones por microorganismos multirresistentes y la coexistencia de factores dependientes del paciente y del tratamiento, lo cual contribuye a situaciones de riesgo para el desarrollo de complicaciones graves. Métodos: Se realizó una revisión exhaustiva del tema en las principales bases de datos de la Biblioteca Virtual de Salud, se utilizó como referencia artículos actualizados publicados principalmente en los últimos 5 años. Análisis y síntesis de la información: Se abordó fundamentalmente el enfoque diagnóstico y terapéutico en los pacientes de alto riesgo de complicaciones por infecciones bacterianas, así como su evaluación integral. Se incluyen herramientas recientes de pesquisa de sepsis y daño orgánico relacionado con ella, que constituyen elementos predictivos de mortalidad en estos pacientes. Conclusiones: El abordaje integral de la neutropenia febril incluye además de una evaluación clínica y humoral exquisita, la aplicación de herramientas pronósticas para la estratificación de riesgo de cada paciente. El tratamiento de los pacientes debe comenzar en la primera hora de haberse documentado la fiebre, ya que las infecciones pueden ser rápidamente progresivas con un alto riesgo de desarrollo de sepsis, inestabilidad hemodinámica y disfunción multiorgánica(AU)


Introduction: Febrile neutropenia frequently affects patients with hematological malignancies and constitutes a medical emergency. Its therapeutic management is a real challenge at present, due to the appearance of infections caused by multiresistant microorganisms and the coexistence of patient- and treatment-dependent factors leading to risk for serious complications. Methods: An exhaustive review was conducted about the topic in the main databases of the Virtual Health Library, for which papers mainly published in the last five years were used as reference. Data analysis and synthesis: A diagnostic and therapeutic approach was applied to the study of patients at high risk for complications due to bacterial infections and their comprehensive evaluation. Recent tools to screen sepsis and sepsis-related organ damage are included which constitute mortality prediction elements in these patients. Conclusions: Comprehensive management of febrile neutropenia includes not only a detailed clinical and humoral evaluation, but also the application of prognostic tools for risk stratification in each patient. Patient treatment should be started within the first hour after fever documentation, since infections may be rapidly progressive with a high risk for the development of sepsis, hemodynamic instability and multiple organ dysfunction(AU)


Subject(s)
Humans , Febrile Neutropenia/diagnosis , Neutropenia/drug therapy , Hematologic Diseases/diagnosis
3.
Rev. chil. pediatr ; 91(4): 597-604, ago. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1138677

ABSTRACT

Resumen: Cada vez es más frecuente la atención médica en la Unidad de Cuidados Intensivos (UCI) de niños o adolescentes inmigrantes como también de aquellos nacidos en nuestro país con padres en tal condición. Esto ha ocasionado, en la actualidad, que el equipo de salud se deba enfrentar con problemas diagnósticos derivados del escaso conocimiento de condiciones genéticas propias de esta población y/o el desarrollo de diversas patologías infrecuentes en nuestro país, algunas resultantes de su condi ción sanitaria. En esta revisión se abordan diversos aspectos de la patología hematológica, infecciosa, parasitaria, respiratoria y cardiovascular, todos tópicos relevantes de conocer durante su estadía en la UCI. Es un deber del equipo de salud actualizarse sobre patologías de baja prevalencia en nuestro país, algunas de ellas muy poco conocidas hasta hace una década, pero que, actualmente, están cada vez más presentes en las UCI del sistema de salud público chileno.


Abstract: It is increasingly common to provide medical care in the Intensive Care Unit (ICU) for immigrant children and adolescents as well as those born in Chile with parents in such condition. Currently, this has caused that the health team has to face diverse infrequent pathologies in our country and/ or diagnostic problems derive from the poor knowledge of genetic conditions of this population, some resulting from their health conditions. This review addresses several aspects of hematological, infectious, parasitic, respiratory, and cardiovascular pathologies, all relevant topics to know during their stay in the ICU. It is a duty of the health team to be updated on pathologies of low prevalence in our country, some of them very little known until a decade ago, but which are currently increasingly present in the ICUs of the Chilean public health system.


Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Respiratory Tract Diseases/diagnosis , Respiratory Tract Diseases/ethnology , Respiratory Tract Diseases/therapy , Cardiovascular Diseases/diagnosis , Cardiovascular Diseases/ethnology , Cardiovascular Diseases/therapy , Critical Care/methods , Emigrants and Immigrants , Hematologic Diseases/diagnosis , Hematologic Diseases/ethnology , Hematologic Diseases/therapy , Infections/diagnosis , Infections/ethnology , Infections/therapy , Intensive Care Units , Chile/epidemiology , Prevalence
7.
Rev. cuba. med. gen. integr ; 34(4): 112-120, oct.-dic. 2018. tab
Article in Spanish | LILACS, CUMED | ID: biblio-1093471

ABSTRACT

Introducción: Las trombofilias son condiciones que se presentan cuando existe una alteración en la fisiología normal del proceso hemostático, estas patologías son una importante causa de eventos tromboembólicos que tienen una repercusión clínica notoria en el estado de salud del paciente. El síndrome de plaquetas pegajosas es una enfermedad procoagulante autosómica dominante que se caracteriza por la agregación anormal de plaquetas como respuesta a diferentes sustancias. Objetivo: Realizar una breve revisión de la literatura que aborde los aspectos generales del síndrome de plaquetas pegajosas y ofrezca al lector una actualización en el tema. Métodos: Se llevó a cabo una búsqueda bibliográfica en las bases de datos Scielo, PubMed, ScienceDirect y Lilacs. Conclusión: El síndrome de plaquetas pegajosas es una causa importante de trombosis no explicada, y a pesar de ser una condición que amenaza la vida del paciente, no ha sido ampliamente estudiada en la literatura científica y es una entidad desconocida por gran parte del personal de la salud(AU)


Introduction: Thrombophilias are conditions that occur when there is an alteration in the normal physiology of the hemostatic process. These pathologies are an important cause of thromboembolic events that have a notorious clinical impact on the health status of the patient. The sticky platelet syndrome is an autosomal dominant procoagulant disease characterized by the abnormal aggregation of platelets as a response to different substances. Objective: To conduct a brief review of the literature addressing the general aspects of the sticky platelet syndrome and offering the reader an update on the subject. Methods: A literature search was carried out in the databases Scielo, PubMed, Science Direct and Lilacs. Conclusion: The sticky platelet syndrome is an important cause of unexplained thrombosis, and although it is a life-threatening condition, it has not been widely studied in the scientific literature and is an entity still unknown to many health care personnel(AU)


Subject(s)
Humans , Male , Female , Thrombophilia/genetics , Hematologic Diseases/diagnosis , Syndrome
8.
Rev. cuba. cir ; 57(1): 10-21, ene.-mar. 2018. tab
Article in Spanish | LILACS | ID: biblio-960342

ABSTRACT

Introducción: la esplenectomía laparoscópica apareció con el desarrollo de la cirugía de mínimo acceso, se conviertió en la técnica preferida para la exéresis del bazo actualmente. Objetivo: evaluar los resultados de la esplenectomía videolaparoscópica en el tratamiento de las enfermedades hematológicas funcionales benignas. Método: se realizó un estudio descriptivo, longitudinal y prospectivo en 86 pacientes con diagnóstico de enfermedades hematológicas funcionales benignas, atendidos en el Hospital Clínico Quirúrgico Hermanos Ameijeiras desde octubre de 1996 hasta diciembre de 2011. Resultados: en la serie predominó el sexo femenino con 70,9 por ciento y la edad media fue de 40 años (37,8 por ciento). Las enfermedades más frecuentes fueron la púrpura trombocitopénica inmunológica (68,6 por ciento), la anemia hemolítica autoinmune con 23,3 por ciento y la esferocitosis hereditaria con 5,8 por ciento. Requirieron preparación preoperatoria 59,3 por ciento de los pacientes con 50,9 por ciento de respuesta efectiva a esta. El tiempo quirúrgico promedio fue 129 minutos. Como accidentes transoperatorios prevalecieron: la ruptura de la bolsa extractora con 12,8 pr ciento y el sangrado del hilio esplénico con 5,8 por ciento. Esto motivó la conversión a cirugía convencional y la exclusión del seguimiento de 6 pacientes. La complicación posoperatoria más frecuente fue el vómito persistente. El seguimiento de los pacientes se realizó desde los 7 días hasta los 2 años en 78 por ciento de los casos, con un tiempo promedio de 518 días. Hubo remisión total de su enfermedad en 82,7 por ciento de los pacientes, remisión parcial en 15,4 por ciento y solamente 1,9 por ciento no presentó remisión. Conclusiones: la esplenectomía videolaparoscópica es una técnica segura y efectiva en el tratamiento de las enfermedades hematológicas autoinmunes en cirujanos con experiencia en Cirugía de Mínimo Acceso de Avanzada(AU)


Introduction: Laparoscopic splenectomy appeared with the development of minimal access surgery. At the moment, it has become the technique preferred for the spleen exeresis. Objective: To evaluate the outcomes of videolaparoscopic splenectomy in the treatment of benign functional hematological diseases. Method: A descriptive, longitudinal and prospective study was carried out with 86 patients diagnosed with benign functional hematological diseases, attended at Hermanos Ameijeiras Clinical Surgical Hospital, from October 1996 to December 2011. Results: In the series, the female sex predominated (70.9 percent) and the average age was 40 years (37.8 percent). The most frequent diseases were immunological thrombocytopenic purpura (68.6 %), autoimmune hemolytic anemia (23.3 percent), and hereditary spherocytosis (5.8 percent). 59.3 percent of patients required a preoperative preparation, with 50.9 percent of effective response to it. The average surgical time was 129 minutes. The prevailing transoperative accidents were: the rupture of the extractor bag (12.8 percent) and the bleeding of the splenic hilum (5.8 percent). This motivated the change to conventional surgery and the exclusion of the follow-up of 6 patients. The most frequent postoperative complication was persistent vomiting. The follow-up of the patients was carried out from 7 days to 2 years in 78 percent of the cases, with an average time of 518 days. There was total remission of their disease in 82.7 percent of the patients, partial remission in 15.4 percent, and only 1.9 percent did not present any remission. Conclusions: Videolaparoscopic splenectomy is a safe and effective technique in the treatment of autoimmune hematological diseases for surgeons with experience in advanced minimal access surgery(AU)


Subject(s)
Humans , Female , Adult , Splenectomy/methods , Laparoscopy/methods , Minimally Invasive Surgical Procedures/statistics & numerical data , Hematologic Diseases/diagnosis , Epidemiology, Descriptive , Prospective Studies , Longitudinal Studies , Postoperative Nausea and Vomiting/complications
9.
Rev. baiana enferm ; 32: e26065, 2018. tab
Article in Portuguese | LILACS, BDENF | ID: biblio-977308

ABSTRACT

Objetivo caracterizar as pessoas assistidas em um Centro de Referência em doença falciforme em uma cidade do estado da Bahia. Método estudo quantitativo, descritivo e retrospectivo, realizado entre agosto de 2016 e fevereiro de 2017. Os dados secundários coletados em 326 prontuários foram processados no STATA, versão 12.0. Resultados prevalência de adultos jovens, do sexo feminino, solteiras, pretas, com renda de até um salário mínimo, não alfabetizadas, protestantes, com até três filhos. As complicações prevalentes foram crise álgica, icterícia e alterações do baço. As medicações mais utilizadas foram ácido fólico, hidroxiureia, ibuprofeno e dipirona. Permaneceram em tratamento 67,79% das pessoas, enquanto 4,60% foram a óbito. Conclusão pessoas com doença falciforme assistidas em um centro de referência possuíam elevado grau de vulnerabilidade e estavam sujeitas à variabilidade clínica.


Objetivo caracterizar a las personas asistidas en un Centro de Referencia tratamiento de la enfermedad falciforme en una ciudad del estado de Bahía. Método estudio cuantitativo, descriptivo y retrospectivo, realizado entre agosto de 2016 y febrero de 2017. Los datos secundarios colectados en 326 prontuarios fueron procesados en el STATA, versión 12.0. Resultados prevalencia de adultos jóvenes, del sexo femenino, solteras, negras, con renta de hasta un salario mínimo, no alfabetizadas, protestantes, con hasta tres hijos. Las complicaciones prevalentes fueron crisis álgicas, ictericia y alteraciones en el bazo. Los medicamentos más utilizados fueron el ácido fólico, hidroxiurea, ibuprofeno y dipirona. Permanecieron en tratamiento 67,79% de las personas, y 4,60% terminaron en óbito. Conclusión las personas con enfermedad falciforme asistidas en un centro de referencia tenían un grado elevado de vulnerabilidad y estaban sujetas a la variabilidad clínica.


Objective to characterize people assisted in a sickle cell disease referral center in a city in the state of Bahia. Method quantitative, descriptive and retrospective study, performed between August 2016 and February 2017. Secondary data collected in 326 medical records were processed in STATA, version 12.0. Results prevalence of young adults, female, single, black, with income of up to one minimum wage, non-literate, Protestant, with up to three children. The prevalent complications were pain, jaundice, and spleen changes. The most commonly used medications were folic acid, hydroxyurea, ibuprofen and dipyrone. 67.79% of the patients remained in treatment, while 4.60% died. Conclusion people with sickle cell disease attended at a referral center had a high degree of vulnerability and were subject to clinical variability.


Subject(s)
Humans , Male , Female , Health Profile , Epidemiology , Anemia, Sickle Cell , Retrospective Studies , Hematologic Diseases/diagnosis
10.
Ann. Univ. Mar. Ngouabi ; 17(1): 33-38, 2017. tab
Article in French | AIM, AIM | ID: biblio-1258833

ABSTRACT

L'épidémiologie des hémopathies lymphoïdes chroniques au Congo n'est pas connue. L'objectif de cette étude est de rapporter la distribution des hémopathies lymphoïdes chronique à Brazzaville.Patients et méthodes : Il s'agi d'une étude transversale descriptive réalisée dans le Service d'Hématologie Clinique du CHU de Brazzaville au Congo. La période étudiée est de 10 ans (du 1er janvier 2006 au 31 décembre 2015). Etaient inclus dans l'étude tous les dossiers de consultation et d'hospitalisation portant le diagnostic d'hémopathie lymphoïdes chroniques.Résultats : 150 cas d'hémopathies lymphoïdes chroniques ont été diagnostiquées durant la période d'étude. Parmi elles, le myélome multiple représentait 52% de la population étudiée (n=78), le lymphome malin non Hodgkinien 22,67% (n=34), le lymphome de Hodgkin et la leucémie lymphoïde chronique respectivement 9,33% (n=14) et la leucémie à tricholeucocyte 6,67% (n=10). La distribution était essentiellement féminine (sex-ratio=0,70). Les pathologies lymphoprolifératives chroniques étaient plus observées dans la tranche d'âge de 45 à 49 ans (66,7%).Conclusion : Les hémopathies lymphoïdes chroniques constituent par leur fréquence un problème sanitaire. Elles plaident pour des études épidémiologiques analytiques afin de mettre en place une politique préventive de celles-ci


Subject(s)
Congo , Hematologic Diseases , Hematologic Diseases/diagnosis , Hematologic Diseases/epidemiology , Lymphoproliferative Disorders
11.
Rev. salud pública ; 18(6): 946-952, nov.-dic. 2016. graf
Article in Spanish | LILACS | ID: biblio-962036

ABSTRACT

RESUMEN Objetivo Establecer la correlación entre los métodos de Wintrobe y de micro-hematocrito Métodos Se tomaron 407 pacientes asistentes a un laboratorio de tercer nivel a quienes en forma simultánea se les realizó las dos pruebas en estudio. Resultados Mediante el un método estadístico de regresión lineal se encontró un coeficiente de correlación de 0,99. Conclusión Ambos métodos pueden contribuir al análisis clínico de los pacientes a quienes se les solicitó la prueba con fines de diagnóstico, control y seguimiento de diferentes patologías.(AU)


ABSTRACT Objective To establish the correlation between the methods of Wintrobe and micro-hematocrit Methods 407 patients attendees were taken laboratory to third level simultaneo usly tests were performed in the study. Results Using a statistical method of linear regression correlation coefficient of 0,99 was found. Conclusion Both methods can contribute to the clinical analysis of patients who were asked to test for diagnosis or control of different pathologies.(AU)


Subject(s)
Humans , Blood Sedimentation , Hematocrit/methods , Hematologic Diseases/diagnosis , Linear Models , Epidemiology, Descriptive
12.
Brasília; Brasil. Ministério da Saúde; 2016. ilus.
Monography in Portuguese | LILACS | ID: biblio-837109

ABSTRACT

Este material tem como objetivo orientar as equipes que atuam na AB, qualificando o processo de referenciamento de usuários para outros serviços especializados. É uma ferramenta, ao mesmo tempo, de gestão e de cuidado, pois tanto guiam as decisões dos profissionais solicitantes quanto se constitui como referência que modula as avaliações apresentadas pelos médicos reguladores.


Subject(s)
Humans , Clinical Protocols/standards , Hematologic Diseases/diagnosis , Hematology/standards , Primary Health Care/standards , Secondary Care/standards , Clinical Laboratory Techniques , Hematologic Diseases/therapy
13.
Article in English | IMSEAR | ID: sea-159392

ABSTRACT

The mouth is a unique site, due to the presence of hard and soft tissues in close approximation. It serves in various purposes of speech, mastication and digestion. It is an important entry point for many pathogens in the body. Many systemic diseases manifest in the oral cavity and mouth can show early signs or the only signs of a disease process at a site elsewhere. As the mouth is an easily accessible site, the indicators it shows of various diseases should not be overlooked. A dentist thus can frequently be exposed to such conditions and play a key role in the diagnostic procedure of various systemic diseases. Appropriate knowledge of these oral manifestations is essential for early diagnosis, treatment and referral of cases.


Subject(s)
Endocrine System Diseases/diagnosis , Gastrointestinal Diseases/diagnosis , Hematologic Diseases/diagnosis , Humans , Metabolic Diseases/diagnosis , Mouth/pathology , Nutritional and Metabolic Diseases/diagnosis , Oral Manifestations/diagnosis , Oral Manifestations/epidemiology , Oral Manifestations/etiology , Rheumatic Heart Disease/diagnosis
14.
Article in English | IMSEAR | ID: sea-157597

ABSTRACT

Bone marrow aspiration (BMA) is much more frequently used than bone marrow biopsy (BMB) in the diagnosis of different haematological disorders. BMB is performed primarily in cases where bone marrow aspiration either fails or gives insufficient information. It is the sole modality of diagnosis in some situations and may give useful information of prognostic importance. The present study was done to find comparative usefulness of each of these procedures in various hematological disorders. Methods: Study subjects included all 45 patients attending the hematology section of pathology department of College of Medicine & Jawaharlal Nehru Memorial Hospital, Kalyani for bone marrow aspiration and who also consented for bone marrow biopsy during the period from 1st July 2011 to 30th June 2012. BMA smears and BMB specimens were obtained from posterior superior iliac spine in the same sitting and stained with Leishman’s stain and Haematoxylin & Eosin stain respectively. Findings of BMA and BMB were compared. Results: BMA was diagnostic in all cases of iron deficiency anemia (IDA), acute myeloid leukemia (AML), chronic myeloid leukemia (CML), chronic lymphocytic leukemia (CLL), idiopathic thrombocytopenic purpura (ITP) and essential thrombocythemia (ET). On the whole, BMA was diagnostic in 82.2% of cases and could accurately reveal hypercellularity in all cases. BMB was the only diagnostic method in myelofibrosis (MF) and provided additional information like fibrosis in cases of AML, CML and MF & pattern of involvement in CLL and multiple myeloma (MM). BMB, however, was poor in assessing iron stores due to leaching of iron during decalcification. Conclusions: BMA alone is sufficient for IDA and ITP. BMB is mandatory for diagnosis of MF and provides additional prognostic information in other cases.


Subject(s)
Biopsy/methods , Biopsy, Needle/methods , Bone Marrow Examination/methods , Hematologic Diseases/classification , Hematologic Diseases/diagnosis , Hematologic Diseases/pathology , Humans , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Purpura, Thrombocytopenic, Idiopathic/pathology , Thrombocythemia, Essential/diagnosis , Thrombocythemia, Essential/pathology
15.
Arch. argent. pediatr ; 112(1): 26-32, feb. 2014. ilus, tab
Article in Spanish | LILACS | ID: lil-708469

ABSTRACT

El síndrome de Kabuki es una entidad génica caracterizada por discapacidad intelectual asociada con múltiples anomalías sistémicas. El diagnóstico es fundamentalmente clínico y se basa en dismorfas faciales típicas, anomalías esqueléticas menores, persistencia de las almohadillas del pulpejo de los dedos y défcit de crecimiento posnatal. Otros hallazgos incluyen cardiopatía congénita, anomalías genitourinarias, fisura de paladar y/o labial, atresia anal y défcits funcionales, como mayor susceptibilidad a infecciones, enfermedades autoinmunes y endocrinológicas e hipoacusia. El objetivo de este trabajo es describir dos pacientes con diagnóstico clínico de síndrome de Kabuki, destacando los hallazgos fenotípicos y malformaciones asociadas.


Kabuki syndrome is a genetic entity with multiple anomalies associated with intellectual disability. The clinical diagnosis is based on typical facial features, minor skeletal abnormalities, fnger pads, and postnatal growth defcit. Other fndings may include congenital heart disease, genitourinary anomalies, oral clefts, anal atresia, increased susceptibility to infections, autoimmune and endocrine disease and hearing loss. The objective of this paper is to describe two patients with clinical diagnosis of Kabuki syndrome, highlighting the phenotypic fndings and associated malformations.


Subject(s)
Child , Child, Preschool , Female , Humans , Abnormalities, Multiple/diagnosis , Face/abnormalities , Hematologic Diseases/diagnosis , Vestibular Diseases/diagnosis , Phenotype
16.
JPMI-Journal of Postgraduate Medical Institute. 2014; 28 (2): 217-221
in English | IMEMR | ID: emr-157724

ABSTRACT

To determine the usefulness of bone marrow aspiration and trephine biopsy in evaluation of the bone marrow in routine haematological practice. This study included 443 cases of bone marrow examination, referred to Pathology Department, Lady Reading Hospital Peshawar during the period extending from January 2012 till July 2013. All the bone marrow smears and bone biopsy sections were examined in detail. The diagnosis and findings on aspirate and biopsy were evaluated and compared with each other. In 73.8% of the cases the bone marrow aspiration and trephine biopsy showed same diagnosis i.e., bone marrow aspiration alone was sufficient for diagnosis in these cases. In the remaining 116 [26.2%] cases trephine biopsy sections or touch imprints were found to be necessary in for making final diagnosis. These cases were those of the hypoplastic / aplastic marrows, Myelofibrosis, lymphomatous infiltration and chronic granulomatous inflammation. The study results suggest that both the aspirate and trephine biopsy complement each other. Nutritional anaemias, Haematological Malignanciesand Immune Thrombocytopenia can be readily diagnosed by bone marrow aspiration alone. Trephine biopsy is necessary for diagnosing Granulomatous Inflammation and Hypoplastic/Aplastic Anaemia. Also trephinebiopsy is required to diagnose Myelofibrosis and Lymphomatous infiltration


Subject(s)
Humans , Biopsy, Needle , Biopsy/methods , Diagnostic Techniques and Procedures , Bone Marrow/pathology , Anemia, Aplastic/pathology , Anemia, Megaloblastic/pathology , Hematologic Diseases/diagnosis , Myelodysplastic Syndromes/pathology
17.
Lima; s.n; 2013. 24 p. tab, graf.
Thesis in Spanish | LIPECS, LILACS, LIPECS | ID: lil-724586

ABSTRACT

Objetivos. Detectar anticuerpos inesperados en pacientes politransfundidos. Identificar las especificidades de los anticuerpos inesperados detectados. Determinar el grupo de enfermedades hematológicas que conllevan al uso de paquetes globulares de forma crónica. Detectar la presencia de reacciones hemolíticas transfusionales. Métodos. Se trata de un estudio descriptivo de corte prospectivo. Un paciente politransfundido se definió como el que ha recibido al menos 5 paquetes globulares en los últimos tres meses, el estudio inmunohematológico se realizó 15 días después de la última transfusión; de resultar positivo el estudio se procedió a la identificación y a determinar el fenotipo de glóbulos rojos. Además se estudió la edad, el sexo, el tiempo de evolución, el número de paquetes globulares transfundidos y reacciones hemolíticas. Se obtuvo un total de 143 pacientes que cumplían estos criterios desde noviembre de 2012 a abril de 2013. Resultados: Durante el periodo de estudio 3618 paquetes globulares, fueron transfundidos en 606 pacientes del servicio de Hematología, de los cuales cumplían los criterios de poli-transfundido 143, 18 de ellos (12.6 por ciento) tuvieron al menos un anticuerpo inesperado detectado, 13 tuvieron anticuerpo único y 5 asociaciones de anticuerpos. La frecuencia de los anticuerpos fue: anti-E (33.3 por ciento), anti-Dia y anti-C (11.1 por ciento cada uno), anti-Jka, anti-e, antí-c (5.6 por ciento cada uno). Entre los pacientes con asociaciones de anticuerpos, 3 de ellos tenían anti-E y en 4 estaban comprometidos antígenos del sistema Rh. El anti-Dia, fue el único anticuerpo de baja incidencia detectado en este estudio, en dos casos como anticuerpo único y en tres casos como asociación de anticuerpos. El fenotipo más frecuente encontrado en este estudio fue C+E-c+e+K-. No se reportaron reacciones hemolíticas. Conclusión: los pacientes politransfundidos tienen alta probabilidad de formar a los anticuerpos a concentrados de paquetes...


Objective: To estimate the frequency of unexpected antibodies in polytransfused patients. Identify unexpected antibody specificities detected. Determine the group of hematologic malignancies that involve the use of red blood cells package chronically. Detecting the presence of hemolytic transfusion reactions. Methods: This is a prospective cross sectional study. A polytransfused patient was defined as the one that has received at least 5 units of blood over the last three months, the study of immune hematology was performed 15 days after the last transfusion, testing positive the study, proceeded to identify and determine red cell phenotype. In addition, we studied the age, sex, time of evolution, the number of units of blood transfused and hemolytic reactions. There were a total of 143 patients who met these criteria from November 2012 to April 2013. Results: During the study period, 3618 packed red blood cells were transfused in 606 patients of the hematology service, of whom met criteria for multiple transfusions 143, 18 of them (12.6 per cent) had at least one unexpected antibody detected, 13 had unique antibody and 5 associations antibodies. The frequency of the antibodies was: anti-E (33.3 per cent), anti-Dia and anti-C (11.1 per cent each), anti-Jka, anti-e, anti-c (5.6 per cent each). Among patients with associations of antibodies, 3 had anti-E and 4 were committed Rh antigens. anti-Dia, was the unique low incidence antibody detected in this study, in both cases as a single antibody in three cases as antibody association. The most common phenotype found in this study was C + E-c + e + K-. Hemolytic reactions were not reported. Conclusion: Polytransfused patients have a high probability of forming antibodies to pack-ages red blood cells, both single and in combination, including antibodies of low incidence, especially in the elderly chronic hematological group transfusion dependent. The number of red blood cells transfused, sex and the evolution time...


Subject(s)
Humans , Male , Adolescent , Adult , Female , Child , Young Adult , Middle Aged , Aged, 80 and over , Antibodies/analysis , Hematologic Diseases/diagnosis , Blood Component Transfusion , Prospective Studies
18.
Medical Forum Monthly. 2012; 23 (5): 61-63
in English | IMEMR | ID: emr-144623

ABSTRACT

Despite the recent advances in the field of hematology in the form of molecular studies and immunophenotyphing, morphological study of bone marrow remains a corner stone in the diagnosis of pediatric hematological diseases. It is also helpful in the diagnosis of many non-hematological diseases. This study is unique in a sense that bone marrow biopsy procedure and morphology reporting were done by a pediatrician trained in clinical hematology. To describe the indications of bone marrow biopsy and frequency of pediatric hematological and non-hematological diseases on morphological basis. This study was conducted at the Pediatric Hematology/Oncology Department, The Children Hospital and the Institute of Child Health Multan from January 2010 to December 2010. This study was conducted on children whether admitted in hematology / oncology ward or referred from various departments of this hospital. A Performa was filled for each patient including detailed history, clinical examination, base line investigation reports and provisional diagnosis. All bone marrow biopsies were performed from posterior iliac spines according to standard protocol for this procedure. Biopsy samples were stained with Leishman stain for morphological study. Bone marrow biopsy report was issued with detailed morphology, morphological diagnosis and suggestion for further investigations e.g. immunophenotyping. Patients age range was 3 months to 13 years with Male: Female = 1:1. Out of 100 bone marrow biopsy reports, disease distribution was acute lymphoblast leukemia [ALL] 30%, acute myeloid leukemia [AML] 7%, lymphoma infiltration 3%, aplastic anemia 18%, idiopathic thrombocytopenic purpra [ITP] 7%, storage disorders 11%, hemolytic anemia 5%, congenital dyserythropoitic anemia [CDA] 2%, red cell aplasia [RCA] 2%, refractory anemia with excessive blasts [RAEB] 2%, nutritional anemia 3%, malaria 3%, reactive changes 5% and normal morphology 2%.In children, acute leukemia is a leading hematological disease on bone marrow morphology followed by aplastic anemia and various non-hematological diseases. Despite availability of advanced diagnostic facilities, bone marrow biopsy is still a useful diagnostic test in many childhood diseases


Subject(s)
Humans , Male , Female , Bone Marrow , Biopsy , Hematologic Diseases/diagnosis , Pediatrics , Leukemia
19.
Rev. Méd. Clín. Condes ; 22(5): 665-676, sept. 2011.
Article in Spanish | LILACS | ID: lil-677272

ABSTRACT

Abordamos algunas de las emergencias oncológicas (complicaciones agudas en el curso evolutivo del cáncer), con acento principal en el diagnóstico. Se tratan las más características, entre estas, las emergencias cardiovasculares (síndrome de Vena Cava superior, taponamiento cardiaco), las emergencias metabólicas (hipercalcemia, lisis tumoral) emergencias neurológicas (compresión medular, hipertensión endocraneana).


We approach some of the oncologic emergencies (acute complications in the evolution of Cancer), with emphasis in diagnosis. This article deals with the most typical ones, including cardiovascular emergencies (Superior Vena Cava Syndrome, Cardiac Tamponade); metabolic emergencies(Hypercalcemia, Tumor Lysis Syndrome) and neurologic emergencies (Spinal Cord Compression, Intracranial Hypertension).


Subject(s)
Humans , Neoplasms/complications , Neoplasms/diagnosis , Emergencies , Cardiovascular Diseases/diagnosis , Nervous System Diseases/diagnosis , Hematologic Diseases/diagnosis , Metabolic Diseases/diagnosis
20.
Article in Spanish | LILACS | ID: lil-615334

ABSTRACT

En los últimos años se ha producido un extraordinario avance en el conocimiento de los complejos mecanismos del proceso hemostático y de los aspectos genéticos, fisiopatológicos, clínicos, diagnósticos y terapéuticos de las enfermedades hemorrágicas y trombóticas. El Departamento de Hemostasia del Instituto de Hematología e Inmunología ha logrado en este último decenio, resultados satisfactorios en la investigación, la asistencia y la docencia. Se han introducido nuevas técnicas para el diagnóstico de enfermedades hemorrágicas y trombóticas, se han ejecutado diferentes proyectos de investigación sobre diferentes temáticas: hemofilia, púrpura trombocitopénica inmune, valoración de aspectos psicosociales, introducción de nuevas terapéutica, entre otras. Se ha impartido docencia en cursos nacionales e internacionales, se ha contribuido a la formación de especialistas en hematología y de otras especialidades, así como de técnicos de laboratorio clínico. Nuestro grupo de trabajo ha brindado asistencia médica continuada a pacientes con tendencia hemorrágica y trombótica y ha participado activamente en la presentación y publicación de trabajos científicos en eventos y revistas nacionales y extranjeras. El desarrollo del Programa de Atención Integral al Hemofílico ha constituido uno de los aspectos relevantes de la actividad del departamento en esta etapa


In past years has been an extraordinary advance in the knowledge of the complex mechanisms of hemostatic process and of the genetic, pathophysiological, clinical, diagnostic and therapeutic features of hemorrhagic and thrombotic diseases. The Department of Hemostasis of the Institute of Hematology and Immunology has achieved in this past ten years satisfactory results in the research, assistance and teaching. New techniques have been introduced for the diagnosis of hemorrhagic and thrombotic diseases applying different research projects on different subject matters: hemophilia, purpura, immune thrombocytopenia, assessment of psychosocial features, and introduction of new therapies, among others. Teaching has been given in national and international courses, contribution to training of specialists in hematology and of other different specialties, as well as of clinic laboratory technicians. Our working group has supplied continued medical assistance to patients with hemorrhagic and thrombotic trend and has been involved in an active way in the presentation and publication of scientific papers in events and national and foreign journals. The development of the Integral Care Program for hemophilic patients has been one of the relevant features of the activity of the department during this stage


Subject(s)
Humans , Male , Female , Hematologic Diseases/diagnosis , Hematologic Diseases/prevention & control , Hemostasis/physiology , Patient Care/methods , Hemophilia A/prevention & control
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