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1.
Artículo en Inglés | MEDLINE | ID: mdl-36381556

RESUMEN

3D morphable models (3DMMs) simultaneously reconstruct facial morphology, expression and pose from 2D images, and thus could be an invaluable tool for capturing and characterizing the face and facial behavior in early childhood. However, 3DMM fitting on infants is a largely unexplored problem. All publicly available 3DMMs are developed for adults, and it is unclear if and to what extent they can be used on videos of infants. In this paper, we compare five state-of-the-art 3DMM fitting methods on data from naturalistic infant-caregiver interactions. Results suggest that it is possible to produce consistent and subject-specific reconstructions of 3D shape identity from multiple frames, but not from a single frame. Qualitative evaluation highlights that facial regions with high texture variation, such as eyes, brows and mouth, are captured with higher accuracy compared to the rest of the face. Thus, even though a 3DMM developed for adults has significant limitations when reconstructing the morphology of the entire facial region of infants, applications that involve analysis of facial behavior can be feasible. Our encouraging results, combined with the unique ability of 3DMMs to disentangle two major sources of noise for expression analysis (i.e., identity bias and pose variations), motivate future research on using 3DMMs to measure the facial behavior of infants.

2.
Mol Autism ; 13(1): 28, 2022 06 27.
Artículo en Inglés | MEDLINE | ID: mdl-35761377

RESUMEN

BACKGROUND: Canonical babbling-producing syllables with a mature consonant, full vowel, and smooth transition-is an important developmental milestone that typically occurs in the first year of life. Some studies indicate delayed or reduced canonical babbling in infants at high familial likelihood for autism spectrum disorder (ASD) or who later receive an ASD diagnosis, but evidence is mixed. More refined characterization of babbling in the first year of life in infants with high likelihood for ASD is needed. METHODS: Vocalizations produced at 6 and 12 months by infants (n = 267) taking part in a longitudinal study were coded for canonical and non-canonical syllables. Infants were categorized as low familial likelihood (LL), high familial likelihood diagnosed with ASD at 24 months (HL-ASD) or not diagnosed (HL-Neg). Language delay was assessed based on 24-month expressive and receptive language scores. Canonical babble ratio (CBR) was calculated by dividing the number of canonical syllables by the number of total syllables. Generalized linear (mixed) models were used to assess the relationship between group membership and CBR, controlling for site, sex, and maternal education. Logistic regression was used to assess whether canonical babbling ratios at 6 and 12 months predict 24-month diagnostic outcome. RESULTS: No diagnostic group differences in CBR were detected at 6 months, but HL-ASD infants produced significantly lower CBR than both the HL-Neg and LL groups at 12 months. HL-Neg infants with language delay also showed reduced CBR at 12 months. Neither 6- nor 12-month CBR was significant predictors of 24-month diagnostic outcome (ASD versus no ASD) in logistic regression. LIMITATIONS: Small numbers of vocalizations produced by infants at 6 months may limit the reliability of CBR estimates. It is not known if results generalize to infants who are not at high familial likelihood, or infants from more diverse racial and socioeconomic backgrounds. CONCLUSIONS: Lower canonical babbling ratios are apparent by the end of the first year of life in ASD regardless of later language delay, but are also observed for infants with later language delay without ASD. Canonical babbling may lack specificity as an early marker when used on its own.


Asunto(s)
Trastorno del Espectro Autista , Trastorno Autístico , Trastornos del Desarrollo del Lenguaje , Trastorno del Espectro Autista/diagnóstico , Humanos , Lactante , Trastornos del Desarrollo del Lenguaje/diagnóstico , Estudios Longitudinales , Reproducibilidad de los Resultados
3.
Annu Int Conf IEEE Eng Med Biol Soc ; 2019: 6888-6891, 2019 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-31947423

RESUMEN

In this paper, we propose multifrequency body impedance measurement technique for monitoring the onset of vagus nerve stimulation. In response to transcutaneous electrical vagus stimulation, changes occur in body fluids i.e. fluid shifts in extracellular and intracellular media that can be assessed by Cole parameters Re and Ri before and after stimulus, by measuring single side impedance spectroscopy (from 3 kHz to 1 MHz) between wrist and ankle. Following a resting period of 5 minutes in supine position, auricular vagus nerve was stimulated for 3 minutes and BIS performed every 10 seconds, on 23-27 years old, 5 healthy subjects. During the stimulation phase, either an increase or a decrease was observed in extracellular and intracellular fluids. In 3 of the subjects, an ECF decrease of 0.02% per kg was compensated by 0.02 to 0.06% per kg increase in ICF; with a correlation coefficient of -0,70 to -0,79; suggesting fluid shifts between ECF and ICF. With the other 2 subjects, both ECF and ICF decreased by about 0.02% per kg. The study was performed with a limited number of participants; to reach statistically meaningful results measurements will be performed over a larger sample size.


Asunto(s)
Estimulación Eléctrica Transcutánea del Nervio , Estimulación del Nervio Vago , Impedancia Eléctrica , Líquido Extracelular , Líquido Intracelular , Nervio Vago
4.
Pediatr Transplant ; 19(3): 307-15, 2015 May.
Artículo en Inglés | MEDLINE | ID: mdl-25661259

RESUMEN

Bu, combined with TDM-guided dosing, is associated with fewer graft failures/relapses and lower toxicity in pediatric HSCT. We aimed this retrospective study for comparison of weight- and age-based dosing in terms of clinical outcomes such as time to engraftment, early complications, EFS, OS, and toxicity profiles in children receiving iv Bu. Sixty-one children who underwent HSCT from April 2010 to February 2013 by means of a Bu-based conditioning regimen and completed 100 days after transplantation at Ankara Children?s Hematology and Oncology Hospital Bone Marrow Transplantation Unit were enrolled in this study. SOS and neutropenic fever occurred more frequently in the weight-based dosing group. We found a statistically significant correlation between Bu dose and the incidence of SOS (r = 0.26, p = 0.04). Multivariate analysis showed only weight-based dosing of Bu was a significant predictor of SOS (HR = 9.46; p = 0.009). However, no relationship was found between two groups in terms of hemorrhagic cystitis, engraftment syndrome, acute or chronic GvHD, time to engraftment, chimerism, TRM, OS, and EFS rates. Weight-based dosing of Bu may cause higher incidence of SOS and early infectious complications at the places where TDM of Bu cannot be performed.


Asunto(s)
Busulfano/administración & dosificación , Trasplante de Células Madre Hematopoyéticas , Infusiones Intravenosas , Adolescente , Factores de Edad , Peso Corporal , Niño , Preescolar , Ciclofosfamida/uso terapéutico , Femenino , Fiebre/etiología , Supervivencia de Injerto , Enfermedad Injerto contra Huésped/etiología , Enfermedades Hematológicas/terapia , Neoplasias Hematológicas/terapia , Humanos , Lactante , Masculino , Análisis Multivariante , Neutropenia/etiología , Recurrencia , Estudios Retrospectivos , Acondicionamiento Pretrasplante , Resultado del Tratamiento
5.
Int J Oral Maxillofac Surg ; 43(8): 1015-21, 2014 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-24656497

RESUMEN

The use of a laser in surgical procedures involving the soft tissues is advantageous due to its sterile and hemostatic nature. Several different lasers are in use for intraoral soft tissue surgery; however, small, efficient, and fibre-coupled lasers are favoured due to the tightly confined nature of the intraoral environment. This study proposes the use of a 1940-nm thulium fibre laser (Tm:fibre laser) for intraoral soft tissue procedures. Its thermal effects when used to make incisions were investigated. This laser was chosen due to its output wavelength, which is absorbed well by water in biological tissues. Lamb tongues were used in the experiments. The laser was coupled to a 600-µm silica fibre and incisions were made in contact mode with a continuous wave. The extent of ablation and coagulation produced were measured at three different speeds, powers, and numbers of passes. The thermal effects of laser power, movement speed, and number of passes on incision depth and ablation efficiency were determined. The Tm:fibre laser is a promising tool for intraoral surgery, with excellent absorption by tissue, good coagulative qualities, and easy to manipulate fibre output. Its use as an incisional tool with very little to no carbonization is shown in this study.


Asunto(s)
Terapia por Láser/instrumentación , Procedimientos Quirúrgicos Orales/instrumentación , Lengua/cirugía , Animales , Modelos Animales , Ovinos , Tulio
6.
Clin Appl Thromb Hemost ; 19(6): 663-7, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-22815318

RESUMEN

The primary objective was to evaluate the response rate of rituximab therapy for children with chronic immune thrombocytopenic purpura (ITP) and Evans syndrome (ES) and immune reconstitution of these children after rituximab therapy. Eleven patients with chronic ITP and 2 with ES between 6 and 18 years of age and platelet count less than 20 × 10(9)/L received rituximab. Overall response (OR) was defined as an increase in platelet count above 50 × 10(9)/L. The mean age of 13 children (9 girls, 4 boys) was 11.2 ± 3.8 years (6-18). One of the patients with ES had been splenectomized; others were not. The patients mean follow-up time was 10.3 ± 9.3 months after rituximab therapy. Two patients achieved complete response, 4 patients achieved partial response, and OR rate was 46% (6 of 13) after therapy. Seven patients have no response. In conclusion, rituximab may be considered prior to splenectomy in children with chronic ITP and ES with an acceptable toxicity profile.


Asunto(s)
Anemia Hemolítica Autoinmune/tratamiento farmacológico , Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Antineoplásicos/uso terapéutico , Púrpura Trombocitopénica Idiopática/tratamiento farmacológico , Trombocitopenia/tratamiento farmacológico , Adolescente , Anemia Hemolítica Autoinmune/sangre , Anemia Hemolítica Autoinmune/inmunología , Anticuerpos Monoclonales de Origen Murino/efectos adversos , Antineoplásicos/administración & dosificación , Antineoplásicos/efectos adversos , Niño , Femenino , Hemostasis/efectos de los fármacos , Humanos , Recuento de Plaquetas , Púrpura Trombocitopénica Idiopática/sangre , Púrpura Trombocitopénica Idiopática/inmunología , Rituximab , Trombocitopenia/sangre , Trombocitopenia/inmunología , Resultado del Tratamiento
7.
Clin Res Hepatol Gastroenterol ; 36(6): 622-7, 2012 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-22705025

RESUMEN

INTRODUCTION: In this retrospective study, we aimed to evaluate preoperative predictive risk factors for development of pouchitis in the ulcerative colitis (UC) patients with ileal pouch-anal anastomosis (IPAA). METHODS: The records of UC patients who underwent IPAA surgery and were under follow-up in the inflammatory bowel disease (IBD) clinic of our hospital between January 1994 and September 2009 were retrieved. Preoperative clinical, biochemical, and endoscopic findings, as well as preoperative endoscopic activity index (EAI), preoperative disease activity index (DAI) and operative characteristics were recorded. Patients with endoscopic, histological and clinical findings consistent with pouchitis were identified. RESULTS: Out of a total of 49 patients who underwent IPAA for UC, pouchitis was identified in 20 (40.8%) of them. Overall, 37 (75.5%) patients had chronic active disease, eight (16.3%) patients had chronic intermittent disease with frequent relapses, and four (8.2%) patients had fulminant colitis prior to surgery. There was a statistically significant difference (P=0.02) among these patients for the development of pouchitis in postoperative period. The mean EAI (10.1 vs. 8.7, P=0.02) and DAI (10.0 vs. 8.6, P<0.01) in patients with pouchitis were significantly higher than that of patients who did not develop pouchitis. Multivariate analysis revealed steroid dependency (P=0.02), and a higher DAI (P=0.02) to be independent risk factors for the development of pouchitis. CONCLUSION: A more severe preoperative clinical course and steroid dependency, as well as higher endoscopic and disease activity scores may be useful as preoperative predictors of subsequent pouchitis in UC patients undergoing IPAA surgery.


Asunto(s)
Colitis Ulcerosa/cirugía , Reservorios Cólicos/efectos adversos , Íleon/cirugía , Reservoritis/epidemiología , Reservoritis/etiología , Adulto , Anastomosis Quirúrgica/efectos adversos , Femenino , Humanos , Masculino , Periodo Preoperatorio , Estudios Retrospectivos , Medición de Riesgo
8.
Int J Lab Hematol ; 33(6): 651-5, 2011 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-21645281

RESUMEN

Asparaginase, an effective drug in the treatment of childhood acute lymphoblastic leukemia (ALL), has become an important component of most childhood ALL regimens during the remission induction or intensification phases of treatment. The incidence range of asparaginase-associated lipid abnormalities that are seen in children is 67-72%. Lipemia causes erroneous results, which uses photometric methods to analyze blood samples. We describe a case of l-asparaginase-associated severe hyperlipidemia with complete blood count abnormalities. Complete blood count analysis was performed with Beckman COULTER(®) GEN·S™ system, which uses the Coulter Volume, Conductivity, Scatter technology to probe hydrodynamically focused cells. Although an expected significant inaccuracy in hemoglobin determination occurred starting from a lipid value of 3450 mg/dl, we observed that triglyceride level was 1466 mg/dl. Complete blood count analysis revealed that exceptionally high hemoglobin, mean corpuscular hemoglobin, and mean corpuscular hemoglobin concentration levels vs. discordant with red blood cell count, mean corpuscular volume, and hematocrit levels. Total leukocyte count altered spontaneously in a wide range, and was checked with blood smear. Platelet count was in expected range (Table 1). Thus, we thought it was a laboratory error, and the patient's follow-up especially for red cell parameters was made by red blood cell and hematocrit values.


Asunto(s)
Técnicas de Laboratorio Clínico/métodos , Pruebas Hematológicas/métodos , Hiperlipidemias/sangre , Leucemia-Linfoma Linfoblástico de Células Precursoras/sangre , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Asparaginasa/efectos adversos , Asparaginasa/uso terapéutico , Recuento de Células Sanguíneas/instrumentación , Recuento de Células Sanguíneas/métodos , Preescolar , Técnicas de Laboratorio Clínico/instrumentación , Femenino , Pruebas Hematológicas/instrumentación , Humanos , Hiperlipidemias/inducido químicamente , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico
9.
Surg Neurol ; 71(1): 121-5; discussion 125, 2009 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-18295847

RESUMEN

BACKGROUND: A different transodontoid screw fixation technique was studied in delayed type II odontoid fractures. This study presents observations on a different transodontoid fixation technique to remove and decrease the amount of sclerotic layers to accelerate the fusion process after the operation. METHODS: Ten cases of chronic type II odontoid fractures were operated on via transodontoid screw fixation between 2000 and 2007 which were admitted 6 weeks or later after the trauma. Four of these 10 delayed patients were operated on using a new anterior transodontoid screw fixation technique, whereas the other 6 delayed patients were operated on using classical anterior transodontoid screw fixation. RESULTS: Four delayed cases with type II odontoid fracture operated on via this new technique had good results throughout the minimum 38 months' follow-up period. We did not observe nonunion, infection, and/or other complications such as vascular or brain-stem injury. CONCLUSION: Transodontoid screw fixation should be considered as a preferable treatment modality. This surgical intervention may be an alternative to conservative treatment even for cases with delayed type II odontoid fractures.


Asunto(s)
Fracturas Óseas/cirugía , Apófisis Odontoides/lesiones , Apófisis Odontoides/cirugía , Dispositivos de Fijación Ortopédica , Procedimientos Ortopédicos/métodos , Accidentes de Tránsito , Adulto , Tornillos Óseos , Femenino , Fracturas Óseas/diagnóstico por imagen , Humanos , Masculino , Persona de Mediana Edad , Apófisis Odontoides/diagnóstico por imagen , Esclerosis/patología , Esclerosis/cirugía , Tomografía Computarizada por Rayos X
10.
Acta Gastroenterol Belg ; 69(3): 255-60, 2006.
Artículo en Inglés | MEDLINE | ID: mdl-17168120

RESUMEN

AIM: The association between inflammatory bowel disease and pulmonary involvement has not been clearly established. The aim of this prospective study was to define the features of pulmonary function tests and high resolution computed tomography in inflammatory bowel disease patients and the relation between these and disease activity. METHOD: Fifty-two patients with inflammatory bowel disease (20 with Crohn's disease and 32 with ulcerative colitis) were enrolled. The standard pulmonary function tests and thorax high resolution computed tomography findings were investigated with respect to inflammatory bowel disease activity. Crohn's disease activity index and the Rachmilewitz endoscopic activity index for ulcerative colitis were used to assess disease activity. Medications used and smoking status were also documented. RESULTS: Among the patients with ulcerative colitis, 6.25% had an obstructive and/or restrictive ventilatory defect compared with 25% of the patients with Crohn's disease. Fifty percent of the patients with ulcerative colitis and 60% of the patients with Crohn's disease showed abnormal findings in high resolution computed tomography. Pulmonary function tests and high resolution computed tomography abnormalities did not differ significantly between Crohn's disease and ulcerative colitis. No significant difference related to inflammatory bowel disease activity was found (P > 0.05). CONCLUSION: Findings of high resolution computed tomography and the pulmonary function tests did not differ between ulcerative colitis and Crohn's disease. Bowel disease activity did not seem to affect these measurements.


Asunto(s)
Colitis Ulcerosa/diagnóstico por imagen , Colitis Ulcerosa/fisiopatología , Enfermedad de Crohn/diagnóstico por imagen , Enfermedad de Crohn/fisiopatología , Tomografía Computarizada por Rayos X , Capacidad Vital , Adulto , Anciano , Anciano de 80 o más Años , Análisis de Varianza , Endoscopía Gastrointestinal , Femenino , Volumen Espiratorio Forzado , Humanos , Enfermedades Pulmonares Obstructivas/diagnóstico por imagen , Enfermedades Pulmonares Obstructivas/fisiopatología , Masculino , Persona de Mediana Edad , Estudios Prospectivos
12.
Acta Gastroenterol Belg ; 67(3): 303-5, 2004.
Artículo en Inglés | MEDLINE | ID: mdl-15587341

RESUMEN

Liver abscess is a quite rare complication in Crohn's disease. Early diagnosis and differentiation of pyogenic abscess from amoebic abscess are as important as the choosing of proper treatment in the management of liver abscess. Herein, 28-year-old man with Crohn's disease developing liver abscess is presented. He was treated with surgical drainage.


Asunto(s)
Enfermedad de Crohn/complicaciones , Absceso Hepático/etiología , Adulto , Drenaje , Humanos , Absceso Hepático/diagnóstico por imagen , Absceso Hepático/cirugía , Masculino , Tomografía Computarizada por Rayos X
13.
Clin Otolaryngol Allied Sci ; 29(1): 80-3, 2004 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-14961857

RESUMEN

The objective of this study is to investigate the effect of normovolaemic haemodilution (anaemia), haemoglobin level and replacement fluids on the survival of local flaps in rats. Fifty male Wistar rats were divided into four study groups and one control group (10 rats for each). In the study groups, 20% or 30% of blood was withdrawn and replaced by either Gelofusine or saline (0.9%). Single 1.5 cm x 3 cm cranially based dorsal random skin flaps consisting of skin and panniculus carnosus were elevated in all rats. No difference was determined between the control and 20% exsanguinated groups regarding flap survival area. There was a statistically significant difference between the flap survival areas of 20% exsanguinated groups and 30% exsanguinated groups. If the blood loss exceeds 30% of total volume and/or haemoglobin (Hb) level is lower than 10.72 g/dL, the risk of flap necrosis increases significantly.


Asunto(s)
Anemia/complicaciones , Supervivencia de Injerto/efectos de los fármacos , Hemodilución/métodos , Sustitutos del Plasma/administración & dosificación , Colgajos Quirúrgicos/irrigación sanguínea , Anemia/sangre , Animales , Fluidoterapia , Gelatina/administración & dosificación , Hemoglobinas/análisis , Hipovolemia/complicaciones , Masculino , Ratas , Ratas Wistar , Cloruro de Sodio/administración & dosificación , Succinatos/administración & dosificación
14.
Leukemia ; 17(2): 328-33, 2003 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-12592331

RESUMEN

Eight-year event-free survival (EFS) was evaluated in 205 patients with acute lymphoblastic leukemia (ALL), to consider the efficacy of high-dose methylprednisolone (HDMP) given during remission induction chemotherapy between 1 and 29 days. The St Jude Total XI Study protocol was used after some minor modifications in this trial. Patients were randomized into two groups. Group A (n = 108) received conventional dose (60 mg/m(2)/day orally) prednisolone and group B (n = 97) received HDMP (Prednol-L, 900-600 mg/m(2) orally) during remission induction chemotherapy. Complete remission was obtained in 95% of the 205 patients who were followed-up for 11 years; median follow-up was 72 months (range 60-129) and 8-year EFS rate was 60% overall (53% in group A, 66% in group B). The EFS rate of group B was significantly higher than of group A (P = 0.05). The 8-year EFS rate of groups A and B in the high-risk groups was 39% vs 63% (P = 0.002). When we compared 8-year EFS rate in groups A and B in the high-risk subgroup for both ages together /=10 years, it was 44% vs 74%, respectively. Among patients in the high-risk subgroup with a WBC count >/=50 x 10(9)/l, the 8-year EFS was 38% in group A vs58% in group B. During the 11-year follow-up period, a total of 64 relapses occurred in 205 patients. In group A relapses were higher (39%) than in group B (23%) (P = 0.05). These results suggest that HDMP during remission-induction chemotherapy improves the EFS rate significantly for high-risk patients in terms of the chances of cure.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Metilprednisolona/uso terapéutico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Prednisolona/uso terapéutico , Adolescente , Niño , Preescolar , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidad , Inducción de Remisión/métodos , Estudios Retrospectivos , Tasa de Supervivencia , Factores de Tiempo
16.
Leuk Res ; 25(8): 647-9, 2001 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-11397468

RESUMEN

The aim of this study was to interpret the antibody response to hepatitis B (HB) vaccination following a two booster dose schedule in 94 acute lymphoblastic leukemia (ALL) patients. All patients were between 1-16 years of age with negative hepatitis B virus (HBV) serology and normal hepatic function. Fifty patients were vaccinated with Engerix B vaccine, and 44 patients were vaccinated with GenHevac B vaccine, with a schedule of 0, 1, 6 and 0, 1, 2, as well as booster doses, in 12 and 6 months respectively. A second booster was given as a fifth dose to 16 unresponsive patients in each vaccine group, 3 and 6 months after the first booster for Engerix B and GenHevac B vaccines respectively. Dosage was 20 microg HbsAg for all patients. Seroconversion rates with protective level antibody were 35.1% (n=33/94). The figures were 32.1% (n=16/50) and 38.6% (n=17/44) for Engerix B and GenHevac B vaccines, respectively. Seroconversion rate in patients younger than 10 years old was found to be higher (39.11%) than older patients (24%), but this was not statistically significant. This study indicates that one third of the leukemic children undergoing maintenence chemotherapy responded to HB vaccine with protective titers of anti-HBs. We recommend HB vaccination especially in developing countries.


Asunto(s)
Vacunas contra Hepatitis B/administración & dosificación , Inmunización Secundaria , Leucemia-Linfoma Linfoblástico de Células Precursoras/inmunología , Vacunas Sintéticas/administración & dosificación , Adolescente , Niño , Preescolar , Femenino , Anticuerpos contra la Hepatitis B/biosíntesis , Anticuerpos contra la Hepatitis B/sangre , Anticuerpos contra la Hepatitis B/inmunología , Vacunas contra Hepatitis B/inmunología , Humanos , Lactante , Masculino , Vacunas Sintéticas/inmunología
17.
Clin Lab Haematol ; 23(1): 33-7, 2001 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-11422228

RESUMEN

The effect of single high-dose methylprednisolone (HDMP) on eosinophil and lymphocyte phenotype in two siblings with marked hypereosinophilia was investigated. Since conventional dose steroids failed to produce a haematological and clinical response in case one, both children were given HDMP (20 mg/kg/day). Eosinophils and lymphocytes showed an activated state before treatment, characterized by marked expression of CD11b, CD18, CD45RO on eosinophils and increased HLA-DR, CD95, CD18, CD38 on lymphocytes. Twenty-four hours after administration of HDMP a dramatic reduction in peripheral blood eosinophil count was observed in both patients associated with phenotypic changes characterized by decreased expression of CD11b, CD18, CD13 and increased CD95 expression in one. Furthermore, HDMP treatment induced a drop in the expression of CD95 and CD18 on lymphocytes. These changes may suggest a suppressive role for HDMP on eosinophil and lymphocyte activation which may have contributed to the haematological and clinical response.


Asunto(s)
Eosinófilos/efectos de los fármacos , Síndrome Hipereosinofílico/tratamiento farmacológico , Síndrome Hipereosinofílico/genética , Linfocitos/efectos de los fármacos , Metilprednisolona/administración & dosificación , Antígenos CD/análisis , Preescolar , Relación Dosis-Respuesta a Droga , Eosinófilos/química , Eosinófilos/patología , Salud de la Familia , Femenino , Citometría de Flujo , Humanos , Síndrome Hipereosinofílico/sangre , Activación de Linfocitos/efectos de los fármacos , Linfocitos/química , Linfocitos/patología , Metilprednisolona/farmacología , Núcleo Familiar , Fenotipo
18.
Thromb Res ; 101(4): 231-4, 2001 Feb 15.
Artículo en Inglés | MEDLINE | ID: mdl-11248283

RESUMEN

Deficiency of plasma platelet-activating factor (PAF) acetylhydrolase resulting from a missense mutation (Val279Phe) in exon 9 of the gene has been described exclusively in the Japanese population with a very high frequency. This study describes the distribution of the mutation in Turkey and two other Turkic nations, Kyrgyzstan in central Asia and Azerbaijan bordering the Caspian Sea. Among 358 unrelated healthy subjects studied from Turkish population, only 3 had the mutation in heterozygous state (0.84%). Family studies also revealed the presence of homozygous individuals in close relatives of one of these subjects. Among 143 healthy subjects studied from Kyrgyzstan, 12 were heterozygous for the mutation (8.4%). No mutation was detected among 100 healthy individuals studied from Azerbaijan. However, it was suggested that the number of subjects was not enough to draw any conclusion about the prevalence of the mutation in the populations studied. Contrary to the previous notions, identification of the mutation in Turkey and Kyrgyzstan shows the existence of the mutation in non-Japanese populations as well.


Asunto(s)
Mutación Missense , Fosfolipasas A/deficiencia , Fosfolipasas A/genética , 1-Alquil-2-acetilglicerofosfocolina Esterasa , Adolescente , Azerbaiyán , Niño , Preescolar , Exones , Femenino , Frecuencia de los Genes , Heterocigoto , Homocigoto , Humanos , Japón , Kirguistán , Masculino , Linaje , Turquía
19.
Pediatr Hematol Oncol ; 18(8): 525-9, 2001 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-11764102

RESUMEN

High-dose methylprednisolone (HDMP) has been shown to induce differentiation of myeloid leukemic cells with a remarkable antileukemic effect in children with various subtypes of acute myeloblastic leukemia (AML). Here the beneficial effect of short-course HDMP therapy in a child with myelodysplastic syndrome (MDS) is reported. Oral methylprednisolone sodium succinate (Prednol-L) was administered at a single daily dose of 30 mg/kg for 5 days to a 4-year-old girl with refractory anemia with excess of blasts and hypocellular bone marrow before the initiation of chemotherapy. In addition to dramatic clinical improvement, the patient's white blood cell count increased from 2.3 x 10(9)/L to 5.0 x 10(9)/L, and peripheral blood blast cells disappeared 4 days after HDMP treatment. Repeated bone marrow aspirate 1 week after the initiation of HDMP disclosed increased cellularity with no blasts. Furthermore, short-course HDMP treatment stimulated the increase in the number of peripheral blood lymphocytes and CD3+, CD4+, CD8+, CD19+, CD34+, and NK cells. Results obtained with HDMP from the previous studies and the present case suggest that high-dose methylprednisolone is a promising agent in the treatment of AIDS and it is recommended as an initial treatment especially for MDS children with hypocellular bone marrow at presentation.


Asunto(s)
Hemisuccinato de Metilprednisolona/administración & dosificación , Síndromes Mielodisplásicos/tratamiento farmacológico , Esteroides/administración & dosificación , Anemia Refractaria con Exceso de Blastos/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Recuento de Células Sanguíneas , Médula Ósea/efectos de los fármacos , Médula Ósea/patología , Preescolar , Femenino , Humanos , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Inducción de Remisión , Resultado del Tratamiento
20.
Pediatr Hematol Oncol ; 18(8): 531-6, 2001 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-11764103

RESUMEN

The authors report a case of hemophagocytic syndrome (HPS) associated with acute visceral leishmaniasis (VL). A 4-year-old boy was admitted with high fever, hepatosplenomegaly, and pancytopenia. Elevated serum ferritin and triglyceride, low fibrinogen levels, and bone-marrow (BM) histiocytic hyperplasia with prominent hemophagocytosis were consistent with a HPS. An initial diagnosis of kala-azar was refuted because of negativity of BM aspiration and serology for this parasite, and the diagnosis HPS was made. Three months after first admission, reevaluation of the BM aspiration revealed many amastigotes of Leishmania parasites. The serology of VL became positive, finally establishing the diagnosis of VL. Although specific therapy for VL was instituted, the patient died 4 weeks after the diagnosis.


Asunto(s)
Histiocitosis de Células no Langerhans/parasitología , Leishmaniasis Visceral/complicaciones , Médula Ósea/parasitología , Preescolar , Enfermedad Crítica , Resultado Fatal , Hemorragia Gastrointestinal/etiología , Humanos , Leishmaniasis Visceral/diagnóstico , Leishmaniasis Visceral/tratamiento farmacológico , Masculino , Infecciones Oportunistas/etiología
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