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1.
Development ; 150(7)2023 04 01.
Artículo en Inglés | MEDLINE | ID: mdl-36897564

RESUMEN

During morphogenesis, large-scale changes of tissue primordia are coordinated across an embryo. In Drosophila, several tissue primordia and embryonic regions are bordered or encircled by supracellular actomyosin cables, junctional actomyosin enrichments networked between many neighbouring cells. We show that the single Drosophila Alp/Enigma-family protein Zasp52, which is most prominently found in Z-discs of muscles, is a component of many supracellular actomyosin structures during embryogenesis, including the ventral midline and the boundary of the salivary gland placode. We reveal that Zasp52 contains within its central coiled-coil region a type of actin-binding motif usually found in CapZbeta proteins, and this domain displays actin-binding activity. Using endogenously-tagged lines, we identify that Zasp52 interacts with junctional components, including APC2, Polychaetoid and Sidekick, and actomyosin regulators. Analysis of zasp52 mutant embryos reveals that the severity of the embryonic defects observed scales inversely with the amount of functional protein left. Large tissue deformations occur where actomyosin cables are found during embryogenesis, and in vivo and in silico analyses suggest a model whereby supracellular Zasp52-containing cables aid to insulate morphogenetic changes from one another.


Asunto(s)
Actomiosina , Proteínas de Drosophila , Animales , Actomiosina/metabolismo , Actinas/metabolismo , Drosophila melanogaster/metabolismo , Proteínas de Drosophila/metabolismo , Drosophila/metabolismo , Sarcómeros/metabolismo , Morfogénesis/genética
2.
J Gerontol Soc Work ; 67(2): 223-229, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-37584083

RESUMEN

Enticing students to expand their knowledge of aging-related issues and careers can be fraught with challenges. Intrinsic and curricula-related factors associated with pursuit of aging-related careers have been identified, but little evidence exists demonstrating the effectiveness of external factors at motivating students to learn more about gerontological practice. This brief report presents findings from a survey of 214 students enrolled in at a single university in the mid-west to assess how likely they would be motivated to learn more about aging by twelve possible incentives with additional opportunities to write in other thoughts. Credit toward required field work, financial incentives such as stipends, scholarships, tuition waivers, and raffles were the most frequently mentioned incentives. Some variation was noted based on race/ethnicity, age, and program of study. Themes emerging from other suggestions provided by students included curricula enhancements, employment incentives, and the suggestion that nothing could entice some students. Findings can be used by scholars in program development and funding requests.


Asunto(s)
Geriatría , Motivación , Humanos , Geriatría/educación , Envejecimiento , Curriculum , Estudiantes
3.
J Med Virol ; 95(12): e29321, 2023 12.
Artículo en Inglés | MEDLINE | ID: mdl-38108193

RESUMEN

In May 2019, a measles outbreak occurred in the French subregion of Loire-Atlantique, particularly affecting Roma settlements. Various obstacles hindered the implementation of postexposure measures among Roma population, resulting in the spread of the cases to other settlements. Suspected cases of measles were immediately investigated and concerned settlements were visited for measles-mumps-rubella (MMR) vaccination. From July 1 to September 3, 2019, a first and then a second Health Reserve team helped for vaccination on the affected and then also the measles-free settlements. Vaccination uptake was monitored with the use of the department's vaccination center immunization registry. Genotyping of selected samples was performed for comparison with viruses circulating at the same time in France and Romania. As of September 16 2019, 109 cases of measles were confirmed among Roma population, including 99 (91%) children under 15 years. Of the 85 people eligible for vaccination, 60 (71%) had not been vaccinated and 23 (27%) had an unknown vaccination status. Sequence comparison revealed that 28/29 sequenced D8 strains were 100% identical to the strain responsible for a large number of cases throughout France in 2019, and to two sequences reported in Romania among sporadic cases. The vaccination campaign resulted in 1136 people on 35 settlements receiving at least one dose of MMR vaccine and in the increase of one-dose MMR vaccine coverage at 24 months from 43% (23/53) to 91% (48/53). With measles transmission continuing in Europe, efforts must be made to meet immunization coverage targets, particularly in hard-to-reach communities where outbreaks may be difficult to control.


Asunto(s)
Sarampión , Paperas , Romaní , Rubéola (Sarampión Alemán) , Niño , Humanos , Brotes de Enfermedades , Francia/epidemiología , Sarampión/epidemiología , Sarampión/prevención & control , Vacuna contra el Sarampión-Parotiditis-Rubéola , Paperas/epidemiología , Rubéola (Sarampión Alemán)/epidemiología , Vacunación
4.
Pediatr Cardiol ; 43(4): 844-854, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-34993558

RESUMEN

Multisystem inflammatory syndrome in children (MIS-C) is one of the most significant sequela of coronavirus disease 2019 (COVID-19) in children. Emerging literature has described myocardial dysfunction in MIS-C patients using traditional and two-dimensional speckle tracking echocardiography in the acute phase. However, data regarding persistence of subclinical myocardial injury after recovery is limited. We aimed to detect these changes with deformation imaging, hypothesizing that left ventricular global longitudinal (GLS) and circumferential strain (GCS) would remain impaired in the chronic phase despite normalization of ventricular function parameters assessed by two-dimensional echocardiography. A retrospective, single-institution review of 22 patients with MIS-C was performed. Fractional shortening, GLS, and GCS, along with regional longitudinal (RLS) and circumferential strain (RCS) were compared across the acute, subacute, and chronic timepoints (presentation, 14-42, and > 42 days, respectively). Mean GLS improved from - 18.4% in the acute phase to - 20.1% in the chronic phase (p = 0.4). Mean GCS improved from - 19.4% in the acute phase to - 23.5% in the chronic phase (p = 0.03). RCS and RLS were impaired in the acute phase and showed a trend towards recovery by the chronic phase, with the exception of the basal anterolateral segment. In our longitudinal study of MIS-C patients, GLS and GCS were lower in the acute phase, corroborating with left ventricular dysfunction by traditional measures. Additionally, as function globally recovers, GLS and GCS also normalize. However, some regional segments continue to have decreased strain values which may be an important subclinical marker for future adverse events.


Asunto(s)
COVID-19 , Disfunción Ventricular Izquierda , COVID-19/complicaciones , Niño , Humanos , Estudios Longitudinales , Reproducibilidad de los Resultados , Estudios Retrospectivos , Síndrome de Respuesta Inflamatoria Sistémica , Disfunción Ventricular Izquierda/diagnóstico por imagen , Función Ventricular Izquierda
5.
Am J Hum Genet ; 99(6): 1388-1394, 2016 Dec 01.
Artículo en Inglés | MEDLINE | ID: mdl-27889061

RESUMEN

Human MITF is, by convention, called the "microphthalmia-associated transcription factor" because of previously published seminal mouse genetic studies; however, mutations in MITF have never been associated with microphthalmia in humans. Here, we describe a syndrome that we term COMMAD, characterized by coloboma, osteopetrosis, microphthalmia, macrocephaly, albinism, and deafness. COMMAD is associated with biallelic MITF mutant alleles and hence suggests a role for MITF in regulating processes such as optic-fissure closure and bone development or homeostasis, which go beyond what is usually seen in individuals carrying monoallelic MITF mutations.


Asunto(s)
Albinismo/genética , Alelos , Coloboma/genética , Sordera/genética , Megalencefalia/genética , Factor de Transcripción Asociado a Microftalmía/genética , Microftalmía/genética , Osteopetrosis/genética , Animales , Preescolar , Femenino , Homocigoto , Humanos , Lactante , Masculino , Linaje , Síndrome , Pez Cebra/embriología , Pez Cebra/genética , Proteínas de Pez Cebra/genética
6.
Epidemiol Infect ; 147: e109, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30869022

RESUMEN

As part of the evaluation of the French plan for the elimination of measles and rubella, we conducted a seroprevalence survey in 2013, aimed at updating seroprevalence data for people 18-32 years old. A secondary objective was to estimate measles incidence in this population during the 2009-2011 outbreak, and thus estimate the exhaustiveness of measles mandatory reporting. We used a cross-sectional survey design, targeting blood donors 18-32 years old, living in France since 2009, who came to give blood in a blood collecting site. We included 4647 people in metropolitan France, 806 people in Réunion Island and 496 in the French Caribbean. A further 3942 individuals were interviewed in the south-east region of metropolitan France to estimate the exhaustiveness of measles mandatory reporting. One of the main findings of this survey is that the proportion of people 18-32 years old susceptible to both measles and rubella infections remained high in France in 2013, 9.2% and 5.4%, respectively, in metropolitan France, even after the promotion campaigns about vaccination catch-up during and following the major measles epidemic in 2009-2011. Applying our results to French census data would suggest that around 1 million people aged 18-32 years old are currently susceptible to measles in France, despite this age group being one of the vaccination targets of the national measles elimination plan. Another important finding is that only an estimated 45% of the true number of cases in this age group was actually notified, despite notification being mandatory.


Asunto(s)
Donantes de Sangre/estadística & datos numéricos , Brotes de Enfermedades , Sarampión/epidemiología , Rubéola (Sarampión Alemán)/epidemiología , Adulto , Susceptibilidad a Enfermedades/epidemiología , Francia/epidemiología , Humanos , Incidencia , Prevalencia , Estudios Seroepidemiológicos , Adulto Joven
7.
Rev Epidemiol Sante Publique ; 67(1): 1-6, 2019 Feb.
Artículo en Francés | MEDLINE | ID: mdl-30514605

RESUMEN

BACKGROUND: France is facing a new resurgence of measles. Since November 2017, the number of cases has been increasing sharply. Immunization coverage in the general population, all ages combined, is below the threshold required for a rapid decline of the virus propagation. Regarding health professionals, the rate of immunization against this disease is insufficiently documented. In this context, the Occupational Health Service of the University Hospital of Caen has carried out an inventory of health personnel knowledge of immunization against measles in the units the most exposed to risk. METHODS: Knowledge of immunization against measles was studied in pediatric, imaging, and pediatric and adult emergencies departments of the University Hospital of Caen, and the Hematology Institute of Lower Normandy (IHBN). The analysis included all health professionals present within these units during the study period: March and April 2018. Data collection was carried out by consulting the medical files of the occupational health unit and considering the set of responses to postal inquiries sent to staff. RESULTS: Measured immunization status data refer to 1017 health professionals. Based on the criteria specific to the recommendations, 234 (50.6%) of the 462 professionals born before 1980 and 437 (78.7%) of the 555 professionals born in or after 1980 could be considered as immune. Of the total sample, 115 (11.3%) had positive measles serology. Among these 1017 professionals, information on the state of immunization against measles was lacking for 174 (17.1%). CONCLUSION: The state of immunization of the nursing staff remains insufficient to prevent the occurrence of measles cases and the staff is also insufficiently informed. It is essential to have knowledge of the immunization status of this population, to organize the vaccination of non-immunized personnel within the occupational health unit, to prevent the emergence of new cases of measles and to reinforce the information regarding the importance of precautions related to airborne transmission in case of measles.


Asunto(s)
Personal de Salud/estadística & datos numéricos , Vacuna Antisarampión/administración & dosificación , Salud Laboral/estadística & datos numéricos , Vacunación/estadística & datos numéricos , Femenino , Francia , Hospitales Universitarios/estadística & datos numéricos , Humanos , Masculino , Sarampión/prevención & control , Factores de Riesgo , Encuestas y Cuestionarios
8.
Br J Clin Pharmacol ; 84(5): 1077-1080, 2018 05.
Artículo en Inglés | MEDLINE | ID: mdl-29520857

RESUMEN

A 30-year-old pregnant female presented with a 2-week history of pityriasis rosea-like eruption. The rash started 2 days after the patient had started taking ondansetron 8 mg for alleviation of moderate-to-severe nausea and vomiting of pregnancy. Physical examination revealed erythematous papulosquamous lesions characterized by annular scaly margins and a dusky centre over the arms, chest, abdomen, lower back and legs. The rash did not involve the palms, sole or mucous membranes, and no lesions were observed on the lymph nodes. Ondansetron was discontinued. The rash ceased to spread and started to disappear within 2 weeks with full resolution noted after 1 month. Analysis of the case using the Naranjo adverse drug reaction probability scale indicated that ondansetron was the probable cause of the pityriasis rosea-like eruption. This is the first case report of pityriasis rosea related to ondansetron therapy.


Asunto(s)
Antieméticos/efectos adversos , Ondansetrón/efectos adversos , Pitiriasis Rosada/inducido químicamente , Complicaciones del Embarazo/inducido químicamente , Adulto , Femenino , Humanos , Embarazo
9.
Mem Inst Oswaldo Cruz ; 113(1): 9-16, 2018 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-29211103

RESUMEN

BACKGROUND: Although first detected in animals, the rare rotavirus strain G10P[14] has been sporadically detected in humans in Slovenia, Thailand, United Kingdom and Australia among other countries. Earlier studies suggest that the strains found in humans resulted from interspecies transmission and reassortment between human and bovine rotavirus strains. OBJECTIVES: In this study, a G10P[14] rotavirus genotype detected in a human stool sample in Honduras during the 2010-2011 rotavirus season, from an unvaccinated 30-month old boy who reported at the hospital with severe diarrhea and vomiting, was characterised to determine the possible evolutionary origin of the rare strain. METHODS: For the sample detected as G10P[14], 10% suspension was prepared and used for RNA extraction and sequence independent amplification. The amplicons were sequenced by next-generation sequencing using the Illumina MiSeq 150 paired end method. The sequence reads were analysed using CLC Genomics Workbench 6.0 and phylogenetic trees were constructed using PhyML version 3.0. FINDINGS: The next generation sequencing and phylogenetic analyses of the 11-segmented genome of the G10P[14] strain allowed classification as G10-P[14]-I2-R2-C2-M2-A3-N2-T6-E2-H3. Six of the genes (VP1, VP2, VP3, VP6, NSP2 and NSP4) were DS-1-like. NSP1 and NSP5 were AU-1-like and NSP3 was T6, which suggests that multiple reassortment events occurred in the evolution of the strain. The phylogenetic analyses and genetic distance calculations showed that the VP7, VP4, VP6, VP1, VP3, NSP1, NSP3 and NSP4 genes clustered predominantly with bovine strains. NSP2 and VP2 genes were most closely related to simian and human strains, respectively, and NSP5 was most closely related to a rhesus strain. MAIN CONCLUSIONS: The genetic characterisation of the G10P[14] strain from Honduras suggests that its genome resulted from multiple reassortment events which were possibly mediated through interspecies transmissions.


Asunto(s)
Virus Reordenados/genética , Infecciones por Rotavirus/virología , Rotavirus/genética , Preescolar , Heces/virología , Genoma Viral , Genotipo , Honduras , Humanos , Masculino , ARN Viral/genética , Rotavirus/aislamiento & purificación , Infecciones por Rotavirus/diagnóstico
10.
Pediatr Cardiol ; 2018 Dec 13.
Artículo en Inglés | MEDLINE | ID: mdl-30547295

RESUMEN

The objective of this study is to identify fetal echocardiographic measures that predict postnatal coarctation of the aorta (CoA). A retrospective review of patients from 2013 to 2017 identified 13 cases of prenatal diagnosis of CoA confirmed postnatally and 14 cases of prenatal diagnosis of CoA with normal arches postnatally. There were 30 controls. Measurements were made and indices applied on all available longitudinal fetal echocardiograms for each patient. Linear mixed effects models were used to examine the between-group differences in the trajectories of the measurements. Significant differences were seen in the true CoA group for the following: smaller distal transverse arch diameter to distance between the left common carotid and left subclavian arteries (DT/LCA-LSCA) index (p = 0.04), smaller distal transverse arch diameter (p = 0.005), and longer brachiocephalic to left common carotid artery (LCA) (p = 0.004) and LCA-left subclavian artery (LSCA) distances (p < 0.0001). Additionally, the LCA/DT index trend appears to differentiate false positives from true coarctations (p < 0.03). The fetal echocardiographic DT/LCA-LSCA index, brachiocephalic-LCA distance and LCA-LSCA distance are significant predictors of postnatal coarctation. The LCA/DT index trend over time may differentiate which of those patients with prenatal concern for coarctation are more likely to develop coarctation postnatally. The use of fetal echocardiographic measures may improve prenatal detection and predication of postnatal coarctation.

11.
J Oncol Pharm Pract ; 23(6): 436-442, 2017 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-27383219

RESUMEN

Purpose Low-dose dopamine has been utilized to improve renal blood flow, urine output, and reduce drug-induced nephrotoxicity. The purpose of this study was to assess changes in renal function, cardiovascular adverse events, and neurologic toxicity in patients receiving cytarabine with or without low-dose dopamine. Methods A retrospective, single-center, cohort study of patients receiving cytarabine at 667 mg/m2/dose or greater, with or without dopamine at ≤5 mcg/kg/min. Cohorts were based upon initiation or absence of low-dose dopamine; cytarabine only, cytarabine + pre- and day of low-dose dopamine, and cytarabine + post-low-dose dopamine. Renal outcomes (urine output, serum creatinine, and creatinine clearance) were compared with baseline and between cohorts. Safety endpoints (arrhythmias, tachycardia, and neurotoxicity) were compared between cohorts based on low-dose dopamine exposure. Results There was no difference in urine output from baseline in all cohorts. Comparing cytarabine only and pre- and day of low-dose dopamine cohorts, there was no difference in urine output. In those receiving low-dose dopamine, there was no difference in serum creatinine and creatinine clearance from baseline. No arrhythmias were documented during the study period, and there was no difference in the incidence of tachycardia between groups (P = 0.66). Neurotoxicity was reported in three patients who were on low-dose dopamine. Conclusion Though variation existed in individual patients administered low-dose dopamine, the use of low-dose dopamine did not significantly impact renal function in this small sample at a single institution. In addition, low-dose dopamine did not negatively impact cardiovascular function.


Asunto(s)
Citarabina/administración & dosificación , Dopamina/administración & dosificación , Adulto , Anciano , Estudios de Cohortes , Femenino , Humanos , Infusiones Intravenosas , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto Joven
12.
Am J Hum Genet ; 92(6): 1001-7, 2013 Jun 06.
Artículo en Inglés | MEDLINE | ID: mdl-23731542

RESUMEN

Infantile myofibromatosis (IM) is a disorder of mesenchymal proliferation characterized by the development of nonmetastasizing tumors in the skin, muscle, bone, and viscera. Occurrence within families across multiple generations is suggestive of an autosomal-dominant (AD) inheritance pattern, but autosomal-recessive (AR) modes of inheritance have also been proposed. We performed whole-exome sequencing (WES) in members of nine unrelated families clinically diagnosed with AD IM to identify the genetic origin of the disorder. In eight of the families, we identified one of two disease-causing mutations, c.1978C>A (p.Pro660Thr) and c.1681C>T (p.Arg561Cys), in PDGFRB. Intriguingly, one family did not have either of these PDGFRB mutations but all affected individuals had a c.4556T>C (p.Leu1519Pro) mutation in NOTCH3. Our studies suggest that mutations in PDGFRB are a cause of IM and highlight NOTCH3 as a candidate gene. Further studies of the crosstalk between PDGFRB and NOTCH pathways may offer new opportunities to identify mutations in other genes that result in IM and is a necessary first step toward understanding the mechanisms of both tumor growth and regression and its targeted treatment.


Asunto(s)
Genes Dominantes , Mutación Missense , Miofibromatosis/congénito , Receptor beta de Factor de Crecimiento Derivado de Plaquetas/genética , Secuencia de Aminoácidos , Secuencia de Bases , Femenino , Estudios de Asociación Genética , Humanos , Masculino , Miofibromatosis/genética , Linaje , Receptor Notch3 , Receptores Notch/genética , Análisis de Secuencia de ADN
13.
N Engl J Med ; 369(12): 1124-33, 2013 Sep 19.
Artículo en Inglés | MEDLINE | ID: mdl-24047061

RESUMEN

BACKGROUND: Physiotherapy involving pelvic-floor muscle training is advocated as first-line treatment for stress urinary incontinence; midurethral-sling surgery is generally recommended when physiotherapy is unsuccessful. Data are lacking from randomized trials comparing these two options as initial therapy. METHODS: We performed a multicenter, randomized trial to compare physiotherapy and midurethral-sling surgery in women with stress urinary incontinence. Crossover between groups was allowed. The primary outcome was subjective improvement, measured by means of the Patient Global Impression of Improvement at 12 months. RESULTS: We randomly assigned 230 women to the surgery group and 230 women to the physiotherapy group. A total of 49.0% of women in the physiotherapy group and 11.2% of women in the surgery group crossed over to the alternative treatment. In an intention-to-treat analysis, subjective improvement was reported by 90.8% of women in the surgery group and 64.4% of women in the physiotherapy group (absolute difference, 26.4 percentage points; 95% confidence interval [CI], 18.1 to 34.5). The rates of subjective cure were 85.2% in the surgery group and 53.4% in the physiotherapy group (absolute difference, 31.8 percentage points; 95% CI, 22.6 to 40.3); rates of objective cure were 76.5% and 58.8%, respectively (absolute difference, 17.8 percentage points; 95% CI, 7.9 to 27.3). A post hoc per-protocol analysis showed that women who crossed over to the surgery group had outcomes similar to those of women initially assigned to surgery and that both these groups had outcomes superior to those of women who did not cross over to surgery. CONCLUSIONS: For women with stress urinary incontinence, initial midurethral-sling surgery, as compared with initial physiotherapy, results in higher rates of subjective improvement and subjective and objective cure at 1 year. (Funded by ZonMw, the Netherlands Organization for Health Research and Development; Dutch Trial Register number, NTR1248.).


Asunto(s)
Modalidades de Fisioterapia , Cabestrillo Suburetral , Incontinencia Urinaria de Esfuerzo/terapia , Anciano , Femenino , Humanos , Análisis de Intención de Tratar , Complicaciones Intraoperatorias , Persona de Mediana Edad , Satisfacción del Paciente , Complicaciones Posoperatorias , Resultado del Tratamiento , Incontinencia Urinaria de Esfuerzo/cirugía
14.
Am J Med Genet A ; 170A(4): 967-77, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-26692240

RESUMEN

We report 13 new individuals with duplications in Xp11.22-p11.23. The index family has one male and two female members in three generations with mild-severe intellectual disability (ID), speech delay, dysmorphic features, early puberty, constipation, and/or hand and foot abnormalities. Affected individuals were found to have two small duplications in Xp11.22 at nucleotide position (hg19) 50,112,063-50,456,458 bp (distal) and 53,160,114-53,713,154 bp (proximal). Collectively, these two regions include 14 RefSeq genes, prompting collection of a larger cohort of patients, in an attempt to delineate critical genes associated with the observed phenotype. In total, we have collected data on nine individuals with duplications overlapping the distal duplication region containing SHROOM4 and DGKK and eight individuals overlapping the proximal region including HUWE1. Duplications of HUWE1 have been previously associated with non-syndromic ID. Our data, with previously published reports, suggest that duplications involving SHROOM4 and DGKK may represent a new syndromic X-linked ID critical region associated with mild to severe ID, speech delay +/- dysarthria, attention deficit disorder, precocious puberty, constipation, and motor delay. We frequently observed foot abnormalities, 5th finger clinodactyly, tapering fingers, constipation, and exercise intolerance in patients with duplications of these two genes. Regarding duplications including the proximal region, our observations agree with previous studies, which have found associations with intellectual disability. In addition, expressive language delay, failure to thrive, motor delay, and 5th finger clinodactyly were also frequently observed in patients with the proximal duplication.


Asunto(s)
Duplicación Cromosómica , Cromosomas Humanos X , Estudios de Asociación Genética , Trastornos de los Cromosomas Sexuales/diagnóstico , Trastornos de los Cromosomas Sexuales/genética , Adolescente , Adulto , Anciano , Niño , Mapeo Cromosómico , Hibridación Genómica Comparativa , Facies , Femenino , Genotipo , Humanos , Masculino , Persona de Mediana Edad , Linaje , Fenotipo , Adulto Joven
15.
J Oncol Pharm Pract ; 22(4): 639-45, 2016 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-26607163

RESUMEN

Blinatumomab is a novel bispecific CD19-directed CD3 T-cell engager recently approved for the treatment of relapsed or refractory Ph-negative acute lymphoblastic leukemia in adults. The drug was approved after a phase II trial in adults with relapsed/refractory disease demonstrated complete remission or hematologic complete remission in 43% of patients within two treatment cycles, of which 40% went on to receive an allogeneic hematopoietic stem cell transplant. In a long-term survival analysis of patients with minimal residual disease after chemotherapy, hematologic relapse-free survival was estimated at 61% at a median of 33 months after blinatumomab. Nine patients underwent hematopoietic stem cell transplant, and six patients remained in complete remission without hematopoietic stem cell transplant or further therapy. Limited data in relapsed pediatric acute lymphoblastic leukemia are reviewed. Blinatumomab carries boxed warnings for neurotoxicity and cytokine release syndrome, which may be serious and lead to treatment interruption and discontinuation. Clinical controversies with blinatumomab include use in patients with Ph-positive acute lymphoblastic leukemia, dosing in underweight adults, and the optimal management of cytokine release syndrome. Oncology pharmacists must be aware of detailed preparation and administration procedures required for safe use of blinatumomab. Clinical trials are ongoing in the first-line setting for patients with Ph-negative acute lymphoblastic leukemia, in Ph-positive acute lymphoblastic leukemia, and other B-cell malignancies.


Asunto(s)
Anticuerpos Biespecíficos/uso terapéutico , Inmunoterapia/métodos , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Anticuerpos Biespecíficos/efectos adversos , Anticuerpos Biespecíficos/farmacología , Humanos , Inducción de Remisión
16.
Fetal Pediatr Pathol ; 35(3): 167-72, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27100475

RESUMEN

INTRODUCTION: Acquired autoimmune myasthenia gravis (MG) is an autoimmune process in which antibodies (AB) directed against the acetylcholine nicotinic receptor (AChR) cause weakness and fatigue of striated muscles. OBJECTIVES: The objective of this study was to determine the range of clinical manifestations in newborns with transient neonatal myasthenia (TNM). METHODS: 62 newborns with mothers who had autoimmune MG were followed by: anthropometric parameters, gestational age, gender, type of delivery completion, Apgar score (AS) in the first and fifth minute, and the emergence of TNM symptoms. RESULTS: For fourteen consecutive years, from a total of 98,000 infants, 62 (0.06%) were born to mothers with autoimmune MG. Four of them (6.4%) had symptoms of TNM. CONCLUSION: Newborns of mothers with MG manifest clinical features of TNM relative to stage of mother's illness. These newborns need monitoring until the seventh day of life.


Asunto(s)
Autoanticuerpos/inmunología , Músculo Esquelético/patología , Miastenia Gravis/patología , Complicaciones del Embarazo/patología , Puntaje de Apgar , Femenino , Humanos , Recién Nacido , Madres , Miastenia Gravis/complicaciones , Miastenia Gravis/diagnóstico , Embarazo , Complicaciones del Embarazo/diagnóstico
17.
Retina ; 35(5): 915-20, 2015 May.
Artículo en Inglés | MEDLINE | ID: mdl-25621945

RESUMEN

PURPOSE: To measure membrane attraction capabilities of enhanced 27-gauge, enhanced 25-gauge, and 23-gauge vitrectomy probes under various parameters. METHODS: A membrane-on-cantilever apparatus was used to measure membrane attraction for enhanced 27-, enhanced 25-, and 23-gauge UltraVit probes (n = 6 for each). The following parameters were evaluated: effects of cut rates and duty cycles on membrane attraction distances, and flow rates and vacuum levels required to attract a membrane at a fixed distance. RESULTS: The enhanced 27-gauge probe had the shortest attraction distance across all cutting speeds and duty cycles. To attract a membrane at a fixed distance, increasing vacuum was necessary with higher cutting rates and smaller probe gauges but flow rate remained relatively constant. The biased open duty cycle was associated with a longer attraction distance than 50/50 or biased closed modes. CONCLUSION: The shorter membrane attraction distance of the enhanced 27-gauge probe versus 23-gauge and enhanced 25-gauge probes may permit greater membrane dissection precision while providing improved access to small tissue planes. Equivalent fluid flow capabilities of the 27-gauge probe compared with the 23-gauge and 25-gauge probes may provide efficient aspiration. Surgeon selection of duty cycle modes may improve intraoperative fluid control and expand the cutter utility as a multifunctional tool.


Asunto(s)
Retina/patología , Vitrectomía/instrumentación , Cuerpo Vítreo/cirugía , Membrana Celular/patología , Humanos , Microcirugia/instrumentación , Modelos Biológicos , Succión , Vacio , Grabación en Video
18.
Pediatr Cardiol ; 36(7): 1452-7, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-25958154

RESUMEN

Smartphone applications that record a single-lead ECG are increasingly available. We sought to determine the utility of a smartphone application (AliveCor) to record supraventricular tachycardia (SVT) and to distinguish atrioventricular reentrant tachycardia (AVRT) from atrioventricular nodal reentrant tachycardia (AVNRT) in pediatric patients. A prior study demonstrated that interpretation of standard event and Holter monitors accurately identifies the tachycardia mechanism in only 45 % of recordings. We performed an IRB-approved prospective study in pediatric patients undergoing an ablation for SVT. Tracings were obtained by placing the smartphone in three different positions on the chest (PI-horizontal, PII-rotated 60° clockwise, and PIII-rotated 120° clockwise). Two blinded pediatric electrophysiologists jointly analyzed a pair of sinus and tachycardia tracings in each position. Tracings with visible retrograde P waves were classified as AVRT. The three positions were compared by Chi-square test. Thirty-seven patients (age 13.7 ± 2.8 years) were enrolled in the study. Twenty-four had AVRT, and 13 had AVNRT. One hundred and eight pairs of tracings were obtained. The correct diagnosis was made in 27/37 (73 %) with position PI, 28/37 (76 %) with PII, and 20/34 (59 %) with PIII (p = 0.04 for PII vs. PIII and p = NS for other comparisons). A single-lead ECG obtained with a smartphone monitor can successfully record SVT in pediatric patients and can predict the SVT mechanism at least as well as previously published reports of Holter monitors, along with the added convenience of not requiring patients to carry a dedicated monitor.


Asunto(s)
Electrocardiografía/métodos , Teléfono Inteligente/instrumentación , Taquicardia por Reentrada en el Nodo Atrioventricular/diagnóstico , Taquicardia Supraventricular/diagnóstico , Adolescente , Niño , Diagnóstico Diferencial , Femenino , Humanos , Masculino , Estudios Prospectivos
19.
Am J Obstet Gynecol ; 211(3): 224.e1-7, 2014 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-24721262

RESUMEN

OBJECTIVE: To determine the clinical utility of hemoglobin level testing in guiding postoperative care following total laparoscopic hysterectomies performed for benign indications. STUDY DESIGN: Retrospective cohort study. RESULTS: A total of 629 women underwent total laparoscopic hysterectomies during the 24 month study period. Only 16 (2.5%) developed symptoms and/or signs suggestive of hemodynamic compromise. When compared to asymptomatic patients, symptomatic patients had a larger decrease in postoperative hemoglobin level (2.66 vs 1.80g/dL, P = .007) and were more likely to undergo blood transfusion, pelvic imaging or reoperation (P < .001). Women with a smaller body mass index and/or higher intraoperative intravenous fluid volume were more likely to have a larger decrease in postoperative hemoglobin level (P < .05). Past surgical history, duration and complexity of the hysterectomy, estimated surgical blood loss, uterine weight, and perioperative use of intravenous ketorolac were not associated with a greater decrease in postoperative hemoglobin (P > .05). Using the University of Pittsburgh Medical Center's annual laparoscopic hysterectomy rate and insurance companies' reimbursement for blood hemoglobin testing, we estimated the national annual cost for hemoglobin testing following total laparoscopic hysterectomy to be $2,804,662. CONCLUSION: Hemoglobin level testing has little clinical benefit following elective total laparoscopic hysterectomy and should be reserved for patients who develop signs or symptoms suggestive of acute anemia. Heath care cost savings can be substantial if this test is no longer routinely requested following total laparoscopic hysterectomies.


Asunto(s)
Hemoglobinas/análisis , Histerectomía/efectos adversos , Laparoscopía/efectos adversos , Cuidados Posoperatorios , Adulto , Índice de Masa Corporal , Estudios de Cohortes , Ahorro de Costo , Femenino , Humanos , Histerectomía/economía , Laparoscopía/economía , Persona de Mediana Edad , Periodo Posoperatorio , Estudios Retrospectivos
20.
J Minim Invasive Gynecol ; 21(4): 596-601, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-24462588

RESUMEN

STUDY OBJECTIVE: To describe a series of intraperitoneal perforated intrauterine contraception devices (IUDs) and to discuss associated findings, methods for diagnosis, and management of this complication. DESIGN: Retrospective review of surgical database between 1998 and 2012 (Canadian Task Force classification II-2). SETTING: University medical center. PATIENTS: Thirty-seven women with a perforated IUD in the intraperitoneal cavity. MEASUREMENTS AND MAIN RESULTS: Nineteen copper IUDs (51%), 17 levonorgestrel-releasing IUDs (LNG-IUDs) (46%), and 1 Lippes loop (3%) were identified. Twenty women (54%) had abdominal pain, 16 (43%) had no symptoms, and 1 (3%) was found to have strings protruding from her anus. Twenty-six women (70%) underwent laparoscopy to remove the IUD, and 6 (16%) underwent hysteroscopy along with laparoscopy. Conversion to laparotomy was required in 4 patients (11%). Two IUDs (5%) caused full-thickness rectouterine fistulas that required laparotomy for repair. Dense adhesions were found in 21 women (57%); and of those, 15 (71%) were associated with a copper IUD. Copper IUDs were significantly more likely than LNG-IUDs to be associated with dense adhesions (p = .02). CONCLUSIONS: Perforated IUDs can be asymptomatic or cause short-term and long-term symptoms. Long-term complications include abscess and fistula formation. Copper IUDs cause a greater inflammatory process than do LNG-IUDs. Even if asymptomatic, we advocate prompt removal of all IUDs that perforate into the peritoneal cavity once they are identified. Laparoscopic surgical removal of an intraperitoneal IUD is a safe and preferred method.


Asunto(s)
Migración de Dispositivo Intrauterino/efectos adversos , Dispositivos Intrauterinos de Cobre/efectos adversos , Perforación Uterina/etiología , Adulto , Bases de Datos Factuales , Femenino , Humanos , Histeroscopía , Dispositivos Intrauterinos/efectos adversos , Dispositivos Intrauterinos Medicados/efectos adversos , Laparoscopía , Fístula Rectal/etiología , Estudios Retrospectivos , Adherencias Tisulares/etiología , Enfermedades Uterinas/etiología , Perforación Uterina/diagnóstico , Perforación Uterina/cirugía , Adulto Joven
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