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1.
Pediatr Dermatol ; 2024 Jul 04.
Artigo em Inglês | MEDLINE | ID: mdl-38961802

RESUMO

We present two pediatric patients who exhibited an unusual clinical presentation of cutaneous acute graft-versus-host disease (GVHD), characterized by livedo-like appearance. Such manifestations of cutaneous acute GVHD have not been previously documented.

2.
JAMA Dermatol ; 160(7): 736-740, 2024 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-38837147

RESUMO

Importance: Detecting activity of morphea can be complex but is crucial for adequate treatment and outcome assessment. The Morphea Activity Measure (MAM) was recently validated, but its responsiveness to change in disease activity has not been studied. Objective: To evaluate the internal and external responsiveness of MAM to changes in disease activity in pediatric patients. Design, Setting, and Participants: This multicenter prospective, longitudinal prognostic study was performed from October 2021 to January 2023 at 4 pediatric referral centers in North America. Consecutive pediatric patients with morphea who were available for data collection at baseline and at a follow-up visit at least 3 months later were studied. Exposure: Patient demographics, clinical characteristics, and measurements of disease activity collected at baseline and the subsequent visit. Main Outcome and Measures: Responsiveness of MAM to disease activity according to the modified Localized Scleroderma Severity Index (mLoSSI), the Physician Global Assessment (PGA), and a patient and parent global assessment (PtGA) was analyzed using mean and percentage change, standardized effect size, and standardized response mean (SRM) from baseline to follow-up 3 or more months later. Differences between patients whose activity improved vs did not improve were evaluated using the Mann-Whitney U test. The correlation between percentage change in MAM score and mLoSSI, the PGA, and the PtGA was calculated using Spearman rank correlation. Results: A total of 43 patients (mean [SD] age at onset, 7.11 [3.18] years; 26 [60.5%] female) were included. The mean change and percentage change in MAM score were significantly larger in those whose disease activity improved by the PGA (mean: -18.75 [95% CI, -31.92 to -5.57] vs 2.73 [95% CI, -1.97 to 7.45]; percentage: -108.08% [95% CI, -155.21% to -60.95%] vs -24.11% [95% CI, -81.22% to 32.99%]) and by mLoSSI (mean: -24.15 [95% CI, -41.89 to -6.41] vs -1.30 [95% CI, -8.50 to 5.70]; percentage: -172.06% [95% CI, -263.68% to -80.45%] vs -21.57% [95% CI, -48.13% to 4.97%]) than in those whose activity did not change. The SRM of MAM was significantly different between groups for both measures; the responsiveness was large in those whose activity decreased by the PGA (-0.75 [95% CI, -1.29 to -0.22]) and mLoSSI (-0.97 [95% CI, -1.69 to -0.25]) and none to small in those whose activity did not change by the PGA (0.11 [95% CI, -0.08 to 0.30]) or mLoSSI (-0.05 [95% CI, -0.34 to 0.23]). Percentage change in MAM score correlated strongly and significantly with change in mLoSSI (ρ = 0.69; P < .001) and PGA (ρ = 0.65; P < .001), but there was no correlation with change in the PtGA (ρ = 0.26; P = .09). Conclusions and Relevance: In this prognostic study, MAM was found to be internally and externally responsive to changes in disease activity. Further evaluation in mixed cohorts of all ages and specialties is needed.


Assuntos
Esclerodermia Localizada , Índice de Gravidade de Doença , Humanos , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/terapia , Feminino , Criança , Masculino , Estudos Prospectivos , Adolescente , Estudos Longitudinais , Prognóstico , Pré-Escolar , Seguimentos
3.
J Am Acad Dermatol ; 91(2): 290-299, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38604489

RESUMO

BACKGROUND: Epidermolysis bullosa (EB), characterized by skin fragility and blistering, often requires hospitalization. Training for inpatient management of EB is limited, with no unified recommendations available in North America. OBJECTIVE: To develop consensus-derived best practices for hands-on inpatient management of EB in both the neonatal and postneonatal period. METHODS: A modified Delphi method (expert-based input via 2 surveys and a final review) was implemented. Available guidelines from EB Clinical Research Consortium centers were analyzed to determine areas of focus and formulate statements to be voted on by EB Clinical Research Consortium members, experienced EB nurses, and select family members. Study participants evaluated statements using a Likert scale: statements with at least 70% agreement were accepted; statements with 30% or more disagreement were rejected. RESULTS: Ten areas of focus were identified. Delphi participants included 15 dermatologists, 8 nurses, and 6 nonhealth care caregivers. Consensus was established on 103/119 neonatal statements and 105/122 postneonatal statements; no statements were rejected. Most recommendations applied to both age groups. LIMITATIONS: Recommendations may require adjustment based on individual patient's clinical context. CONCLUSION: Using the Delphi method, a consensus-derived resource for hospital-based health care professionals who manage patients with EB has been developed to improve the quality of inpatient care.


Assuntos
Consenso , Técnica Delphi , Epidermólise Bolhosa , Humanos , Recém-Nascido , Epidermólise Bolhosa/terapia , Hospitalização , Guias de Prática Clínica como Assunto , Lactente , Feminino , Dermatologia/métodos , Dermatologia/normas , Masculino
5.
Am J Biol Anthropol ; 184(2): e24903, 2024 06.
Artigo em Inglês | MEDLINE | ID: mdl-38308451

RESUMO

OBJECTIVES: Determine the geographic place of origin and maternal lineage of prehistoric human skeletal remains discovered in Puyil Cave, Tabasco State, Mexico, located in a region currently populated by Olmec, Zoque and Maya populations. MATERIALS AND METHODS: All specimens were radiocarbon (14C) dated (beta analytic), had dental modifications classified, and had an analysis of 13 homologous reference points conducted to evaluate artificial cranial deformation (ACD). Following DNA purification, hypervariable region I (HVR-1) of the mitogenome was amplified and Sanger sequenced. Finally, Next Generation Sequencing (NGS) was performed for total DNA. Mitochondrial DNA (mtDNA) variants and haplogroups were determined using BioEdit 7.2 and IGV software and confirmed with MITOMASTER and WebHome softwares. RESULTS: Radiocarbon dating (14C) demonstrated that the inhabitants of Puyil Cave lived during the Archaic and Classic Periods and displayed tabular oblique and tabular mimetic ACD. These pre-Hispanic remains exhibited five mtDNA lineages: A, A2, C1, C1c and D4. Network analysis revealed a close genetic affinity between pre-Hispanic Puyil Cave inhabitants and contemporary Maya subpopulations from Mexico and Guatemala, as well as individuals from Bolivia, Brazil, the Dominican Republic, and China. CONCLUSIONS: Our results elucidate the dispersal of pre-Hispanic Olmec and Maya ancestors and suggest that ACD practices are closely related to Olmec and Maya practices. Additionally, we conclude that ACD has likely been practiced in the region since the Middle-Archaic Period.


Assuntos
Restos Mortais , Cavernas , DNA Mitocondrial , Humanos , México , DNA Mitocondrial/genética , Restos Mortais/química , Restos Mortais/anatomia & histologia , Datação Radiométrica , Masculino , História Antiga , Feminino , Antropologia Física , Arqueologia
6.
Br J Haematol ; 204(4): 1529-1535, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38411250

RESUMO

Chronic myelomonocytic leukaemia (CMML) is a rare haematological disorder characterized by monocytosis and dysplastic changes in myeloid cell lineages. Accurate risk stratification is essential for guiding treatment decisions and assessing prognosis. This study aimed to validate the Artificial Intelligence Prognostic Scoring System for Myelodysplastic Syndromes (AIPSS-MDS) in CMML and to assess its performance compared with traditional scores using data from a Spanish registry (n = 1343) and a Taiwanese hospital (n = 75). In the Spanish cohort, the AIPSS-MDS accurately predicted overall survival (OS) and leukaemia-free survival (LFS), outperforming the Revised-IPSS score. Similarly, in the Taiwanese cohort, the AIPSS-MDS demonstrated accurate predictions for OS and LFS, showing superiority over the IPSS score and performing better than the CPSS and molecular CPSS scores in differentiating patient outcomes. The consistent performance of the AIPSS-MDS across both cohorts highlights its generalizability. Its adoption as a valuable tool for personalized treatment decision-making in CMML enables clinicians to identify high-risk patients who may benefit from different therapeutic interventions. Future studies should explore the integration of genetic information into the AIPSS-MDS to further refine risk stratification in CMML and improve patient outcomes.


Assuntos
Leucemia Mielomonocítica Crônica , Leucemia , Síndromes Mielodisplásicas , Humanos , Leucemia Mielomonocítica Crônica/diagnóstico , Leucemia Mielomonocítica Crônica/genética , Leucemia Mielomonocítica Crônica/tratamento farmacológico , Prognóstico , Inteligência Artificial , Síndromes Mielodisplásicas/terapia , Síndromes Mielodisplásicas/tratamento farmacológico , Medição de Risco
7.
Hemasphere ; 7(10): e961, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37841754

RESUMO

Myelodysplastic neoplasms (MDS) are a heterogeneous group of hematological stem cell disorders characterized by dysplasia, cytopenias, and increased risk of acute leukemia. As prognosis differs widely between patients, and treatment options vary from observation to allogeneic stem cell transplantation, accurate and precise disease risk prognostication is critical for decision making. With this aim, we retrieved registry data from MDS patients from 90 Spanish institutions. A total of 7202 patients were included, which were divided into a training (80%) and a test (20%) set. A machine learning technique (random survival forests) was used to model overall survival (OS) and leukemia-free survival (LFS). The optimal model was based on 8 variables (age, gender, hemoglobin, leukocyte count, platelet count, neutrophil percentage, bone marrow blast, and cytogenetic risk group). This model achieved high accuracy in predicting OS (c-indexes; 0.759 and 0.776) and LFS (c-indexes; 0.812 and 0.845). Importantly, the model was superior to the revised International Prognostic Scoring System (IPSS-R) and the age-adjusted IPSS-R. This difference persisted in different age ranges and in all evaluated disease subgroups. Finally, we validated our results in an external cohort, confirming the superiority of the Artificial Intelligence Prognostic Scoring System for MDS (AIPSS-MDS) over the IPSS-R, and achieving a similar performance as the molecular IPSS. In conclusion, the AIPSS-MDS score is a new prognostic model based exclusively on traditional clinical, hematological, and cytogenetic variables. AIPSS-MDS has a high prognostic accuracy in predicting survival in MDS patients, outperforming other well-established risk-scoring systems.

8.
Rev. Fac. Med. UNAM ; 66(4): 20-25, jul.-ago. 2023. graf
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1514819

RESUMO

Resumen El síndrome de kwashiorkor se caracteriza por malnutrición proteica y edema generalizado; algunos de los factores de riesgo que se asocian a su aparición son: vivir en pobreza, el destete reciente, las infecciones y las dietas basadas en maíz y arroz. Este síndrome puede generar manifestaciones cutáneas como piel delgada y seca, hiperpigmentación, áreas confluentes de descamación, cabello seco, hipopigmentado y desprendible, así como una dermatitis erosiva con predominio en pliegues cutáneos. El diagnóstico se basa principalmente en una evaluación nutricional integral, exploración física y estudios de laboratorio, y el éxito del tratamiento se basa en la rehabilitación nutricional temprana. Caso clínico: lactante del sexo femenino de 8 meses de edad, que acudió al Instituto Nacional de Pediatría (INP), por presentar una dermatosis generalizada de tipo descamativa de 1 mes de evolución, que fue tratada con ketoconazol tópico. Al interrogatorio la madre refiere alimentación exclusiva con atole de maíz por un diagnóstico de "alergia a la leche" y falta de recursos económicos para comprar la fórmula hidrolizada. La paciente presentaba una dermatosis diseminada que afectaba todos los segmentos corporales, caracterizada por placas hiperpigmentadas, bien definidas, de forma irregular, con descamación en láminas gruesas en región perioral y extremidades, así como áreas erosionadas, pálidas y edema generalizado en extremidades. Se realizaron exámenes de laboratorio que mostraron que la paciente tenía anemia (Hb 11.2 g/dL) e hipoalbuminemia (3.3 g/dL) que, en conjunto con las manifestaciones clínicas, integraron el diagnóstico de síndrome de Kwashiorkor. Se informaron los hallazgos clínicos y de laboratorio al servicio de Gastroenterología y Nutrición, quienes realizaron una valoración nutricional integral y decidieron iniciar tratamiento nutricional; por parte del servicio de Dermatología, se indicó el uso de emolientes y cuidados generales de la piel. Veinte días después la dermatosis y el edema habían remitido.


Abstract Kwashiorkor syndrome is characterized by protein malnutrition and edema, risk factors are recent weaning, infections, and diets based on corn and rice. This malnutrition can lead to skin manifestations such as thin, dry skin, hyperpigmentation, confluent areas of scaling, dry, hypopigmented, and detachable hair, as well as erosive dermatitis, predominantly in skin folds. The diagnosis is based on a nutritional evaluation exam, physical examination and laboratory finding, the treatment is based on early nutritional rehabilitation. Clinical case: 8-month-old female infant who attended the Instituto Nacional de Pediatría, presenting a scaling dermatosis of 1 month's evolution that was treated with topical ketoconazole. The mother reported exclusive feeding with corn gruel due to the diagnosis of "lactose allergy" and commented not enough resources to buy hydrolyzed formula. The patient presented a disseminated dermatosis to all body segments, characterized by well-defined, irregularly shaped, hyperpigmented plaques with scaling in thick sheets in the perioral region and extremities, as well as areas of eroded skin and paleness and edema of extremities. Laboratory tests were taken, where anemia (Hb 11.2 g/dl) and hipoalbuminemia (3.3 g/dl) were documented, the diagnosis of kwashiorkor syndrome was integrated. The clinical and laboratory findings were reported to the Gastroenterology and Nutrition service, who performed a nutritional assessment, and began nutritional treatment, emollients and general skin care were documented; twenty days later, the dermatosis and edema had subsided.

9.
JAMA Dermatol ; 159(3): 299-307, 2023 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-36753150

RESUMO

Importance: Morphea is an insidious inflammatory disorder of the skin and deeper tissues. Determining disease activity is challenging yet important to medical decision-making and patient outcomes. Objective: To develop and validate a scoring tool, the Morphea Activity Measure (MAM), to evaluate morphea disease activity of any type or severity that is easy to use in clinical and research settings. Design, Setting, and Participants: This pilot diagnostic study was conducted from September 9, 2019, to March 6, 2020, in 2 phases: development and validation. During the development phase, 14 morphea experts (dermatologists and pediatric dermatologists) used a Delphi consensus method to determine items that would be included in the MAM. The validation phase included 8 investigators who evaluated the tool in collaboration with 14 patients with pediatric morphea (recruited from a referral center [Medical College of Wisconsin]) during a 1-day in-person meeting on March 6, 2020. Main Outcomes and Measures: During the development phase, online survey items were evaluated by experts in morphea using a Likert scale (score range, 0-10, with 0 indicating not important and 10 indicating very important); agreement was defined as a median score of 7.0 or higher, disagreement as a median score of 3.9 or lower, and no consensus as a median score of 4.0 to 6.9. During the validation phase, reliability (interrater and intrarater agreement using intraclass correlation coefficients), validity (using the content validity index and κ statistics as well as correlations with the modified Localized Scleroderma Severity Index and the Physician Global Assessment of Activity using Spearman ρ coefficients), and viability (using qualitative interviews of investigators who used the MAM tool) were evaluated. Descriptive statistics were used for quantitative variables. Data on race and ethnicity categories were collected but not analyzed because skin color was more relevant for the purposes of this study. Results: Among 14 survey respondents during the development phase, 9 (64.3%) were pediatric dermatologists and 5 (35.7%) were dermatologists. After 2 rounds, a final tool was developed comprising 10 items that experts agreed were indicative of morphea activity (new lesion in the past 3 months, enlarging lesion in the past 3 months, linear lesion developing progressive atrophy in the past 3 months, erythema, violaceous rim or color, warmth to the touch, induration, white-yellow or waxy appearance, shiny white wrinkling, and body surface area). The validation phase was conducted with 14 patients (median age, 14.5 years [range, 8.0-18.0 years]; 8 [57.1%] female), 2 dermatologists, and 6 pediatric dermatologists. Interrater and intrarater agreement for MAM total scores was good, with intraclass correlation coefficients of 0.844 (95% CI, 0.681-0.942) for interrater agreement and 0.856 (95% CI, 0.791-0.901) for intrarater agreement. Correlations between the MAM and the modified Localized Scleroderma Severity Index (Spearman ρ = 0.747; P < .001) and the MAM and the Physician Global Assessment of Activity (Spearman ρ = 0.729; P < .001) were moderately strong. In qualitative interviews, evaluators agreed that the tool was easy to use, measured morphea disease activity at a single time point, and should be responsive to changes in morphea disease activity over multiple time points. Conclusions and Relevance: In this study, the MAM was found to be a reliable, valid, and viable tool to measure pediatric morphea activity. Further testing to assess validity in adults and responsiveness to change is needed.


Assuntos
Médicos , Esclerodermia Localizada , Adulto , Humanos , Criança , Feminino , Adolescente , Masculino , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/patologia , Reprodutibilidade dos Testes , Índice de Gravidade de Doença , Pele/patologia
10.
Children (Basel) ; 10(1)2023 Jan 03.
Artigo em Inglês | MEDLINE | ID: mdl-36670646

RESUMO

The aim of this study was to study different 'healthy profiles' through the impact of multiple lifestyle behaviours (sleep patterns, screen time and quality diet) on active commuting to school (ACS) in adolescents. Sixteen secondary schools from four Spanish cities were randomly selected. All participants filled in an "Ad-Hoc" questionnaire to measure their mode of commuting and distance from home to school and their lifestyle behaviours. A multivariate logistic regression model was performed to analyse the main predictor variables of ACS. The final sample was 301 adolescents (50.2% girls; mean age ± SD: 14.9 ± 0.48 years). The percentage of ACS was 64.5%. Multiple logistic regressions showed: boys were more active commuters than girls [OR = 2.28 (CI 95%: 1.12−4.64); p = 0.02]; adolescents who lived farther had lower probability to ACS [OR = 0.74 (CI 95%: 0.69−0.80); p < 0.001]; adolescents who met sleep duration recommendations were more likely to ACS [OR = 3.05 (CI 95%: 1.07−8.69); p = 0.04], while with each hour of sleep, the odds of ACS was reduced [OR = 0.51 (CI 95%: 0.30−0.89); p = 0.02]; higher odds were shown to ACS in adolescents who have more adherence to MD [OR = 1.16(CI 95%: 1.00−1.33); p = 0.05]; and habitual breakfast consumption was inversely associated with ACS [OR = 0.41 (CI: 95%: 0.18−0.96); p = 0.04]. ACS was associated with being a boy, living at a shorter distance to school, a daily sleep time ≥ 8 h and presented a higher adherence to MD.

11.
Pediatr Dermatol ; 40(3): 440-445, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36716766

RESUMO

BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a locally aggressive and potentially lethal vascular tumor of infancy. Current consensus recommendations include the use of vincristine and/or systemic steroids as first-line treatment. Mammalian target of rapamycin (mTOR) inhibitors represent a promising therapy for patients with KHE. The goal of our study is to critically assess the existing literature on outcomes of patients with KHE treated with mTOR inhibitors. METHODS: We conducted a literature search from 1 January 2000, to 30 April 2022. Articles reporting outcomes of patients treated with mTOR inhibitors for KHE were included. Descriptive statistics were used to describe and summarize the outcomes of the treatment. RESULTS: We included 327 patients with a mean age at diagnosis of 9.1 months (SD ± 9). Patients were treated with an mTOR inhibitor for a mean of 15.2 months (SD ± 4.1). A total of 315 (96.3%) patients had positive outcomes including improvement of the tumor size, symptoms and/or laboratory parameters in 227 (85%) and complete remission in 38 (12%). Seven (2%) patients did not respond to treatment and seven (2%) died of sepsis (4), Kasabach-Merritt phenomenon complications (1), cardiac and liver failure due to ductus arteriosus (1), or metastatic disease (1). CONCLUSION: This systematic review supports the efficacy and safety of mTOR inhibitors for KHE. Their use resulted in positive outcomes in terms of decreased symptoms, reduction in tumor size and improvement in biochemical parameters with a mortality rate of 2%. According to these findings, we suggest revised consensus treatment guidelines for KHE with mTOR inhibitors potentially considered first-line therapy.


Assuntos
Hemangioendotelioma , Síndrome de Kasabach-Merritt , Sarcoma de Kaposi , Humanos , Lactente , Síndrome de Kasabach-Merritt/diagnóstico , Sirolimo/uso terapêutico , Inibidores de MTOR , Hemangioendotelioma/diagnóstico , Sarcoma de Kaposi/complicações , Serina-Treonina Quinases TOR/uso terapêutico
12.
Pediatr Dermatol ; 40(3): 422-427, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36579717

RESUMO

BACKGROUND/OBJECTIVES: The primary objective was to assess pain catastrophizing and functional disability in pediatric patients with epidermolysis bullosa (EB) and their parents/guardians. Secondary objectives included examining relationships between pain catastrophizing, functional disability, and correlations with other factors (e.g., age, disease severity, and percent of body surface area (BSA) involved). METHODS: Patients with EB ages 8-16 and their parents/guardians who were English or Spanish speaking completed a one-time online survey. Parent measures included: demographics questionnaire, Pain Catastrophizing Scale-Parent (PCS), and Parent Functional Disability Inventory (FDI). Child measures included: PCS child and child FDI. Higher scores on both scales indicate higher levels of catastrophizing and functional disability. RESULTS: Of 31 children, the mean age was 11.47 years and the majority (70.97%) had dystrophic EB. Mean scores were: 35.84 = PCS parent; 34.58 = PCS child; 30.87 = parent FDI; 29.77 = child FDI. Total scores for PCS parent, parent FDI, and child FDI increased significantly with disease severity and percentage of involved BSA (p < .01 for all). Total scores for PCS child increased significantly with percent of EB skin involvement (p = .04) but not disease severity. Older children reported more functional disability than their parents and younger children (p = .02). CONCLUSIONS: Our results demonstrate significant positive correlations between negative thoughts related to pain and the experience of functional difficulties in patients with EB and their caregivers. Psychological, psychiatric, and/or behavioral interventions to help managing chronic pain may be effective for patients with EB.


Assuntos
Dor Crônica , Epidermólise Bolhosa , Criança , Humanos , Adolescente , Pais/psicologia , Inquéritos e Questionários , Epidermólise Bolhosa/complicações , Catastrofização/psicologia
13.
Blood ; 141(6): 567-578, 2023 02 09.
Artigo em Inglês | MEDLINE | ID: mdl-36399715

RESUMO

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with historically poor outcomes and no worldwide consensus treatment approach. Unique among most hematologic malignancies for its frequent cutaneous involvement, BPDCN can also invade other extramedullary compartments, including the central nervous system. Generally affecting older adults, many patients are unfit to receive intensive chemotherapy, and although hematopoietic stem cell transplantation is preferred for younger, fit individuals, not all are eligible. One recent therapeutic breakthrough is that all BPDCNs express CD123 (IL3Rα) and that this accessible surface marker can be pharmacologically targeted. The first-in-class agent for BPDCN, tagraxofusp, which targets CD123, was approved in December 2018 in the United States for patients with BPDCN aged ≥2 years. Despite favorable response rates in the frontline setting, many patients still relapse in the setting of monotherapy, and outcomes in patients with relapsed/refractory BPDCN remain dismal. Therefore, novel approaches targeting both CD123 and other targets are actively being investigated. To begin to formally address the state of the field, we formed a new collaborative initiative, the North American BPDCN Consortium (NABC). This group of experts, which includes a multidisciplinary panel of hematologists/oncologists, hematopoietic stem cell transplant physicians, pathologists, dermatologists, and pediatric oncologists, was tasked with defining the current standard of care in the field and identifying the most important research questions and future directions in BPDCN. The position findings of the NABC's inaugural meetings are presented herein.


Assuntos
Neoplasias Hematológicas , Transtornos Mieloproliferativos , Neoplasias Cutâneas , Criança , Humanos , Idoso , Padrão de Cuidado , Subunidade alfa de Receptor de Interleucina-3 , Células Dendríticas/patologia , Recidiva Local de Neoplasia/patologia , Transtornos Mieloproliferativos/patologia , Neoplasias Hematológicas/patologia , Neoplasias Cutâneas/patologia , Doença Aguda , América do Norte
14.
Cells ; 11(24)2022 12 12.
Artigo em Inglês | MEDLINE | ID: mdl-36552774

RESUMO

The one-step nucleic acid amplification (OSNA) method allows for the quantitative evaluation of the tumor burden in resected lymph nodes (LNs) in patients with lung cancer. This technique enables to detect macro and micrometastases, facilitating the correct classification of patients for appropriate follow-up of the disease after surgery. Of 160 patients with resectable lung cancer whose LNs were examined by OSNA, H&E and CK19 IHC between July 2015 and December 2018, 110 patients with clinical stages from IA1 to IIIB were selected for follow-up. LN staging in lung cancer by pathological study led to understaging in 13.64% of the cases studied. OSNA allowed to quantify the tumor burden and establish a prognostic value. Patients with a total tumor load of ≥1650 cCP/uL were associated with a significantly increased likelihood of recurrence. Moreover, the survival of patients with <4405 cCP/uL was significantly higher than patients with ≥4405 cCP/uL. The OSNA assay is a rapid and accurate technique for quantifying the tumor burden in the LNs of lung cancer patients and OSNA quantitative data could allow to establish prognostic values for recurrence-free survival and overall survival in this type of malignancy.


Assuntos
Relevância Clínica , Neoplasias Pulmonares , Humanos , Metástase Linfática/patologia , Prognóstico , Linfonodos/patologia , RNA Mensageiro/análise , Biomarcadores Tumorais/análise , Queratina-19/genética , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/patologia
15.
Skinmed ; 20(6): 460-462, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36537683

RESUMO

A 7-year-old girl presented with a 2-year history of recurrent blisters on the skin and oral mucosa. The patient was otherwise healthy, and her family history was unremarkable for any dermatologic or other medical disease. Examination revealed multiple tense vesicles, milia, and atrophic scars present over the extensor surface of the extremities and erosions on the oral mucosa (Figure 1). A skin biopsy established a pauci-inflammatory subepidermal blister (Figure 2a). Direct immunofluorescence (DIF) evidenced the linear deposition of immunoglobulin G (IgG), immunoglobulin M (IgM), and κ and λ chains at the dermal-epithelial junction (DEJ). Indirect immunofluorescence (IIF), using the salt-split technique, established anti-epithelial antibodies on the dermal side (Figure 2b). An enzyme-linked immunosorbent assay (ELISA) was positive for Collagen Type VII (COL7) antibodies. A diagnosis of epidermolysis bullosa acquisita (EBA) was made, and treatment with azathioprine and deflazacort was administered for 8 months with progressive lessening of her symptomatology and complete clinical response at 2-year follow-up. (SKINmed. 2022;20:460-462).


Assuntos
Doenças Autoimunes , Epidermólise Bolhosa Adquirida , Feminino , Humanos , Criança , Vesícula , Pele/patologia , Doenças Autoimunes/patologia , Imunoglobulina G
16.
Indoor Air ; 32(11): e13144, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-36437669

RESUMO

Indoor air in residential dwellings can contain a variety of chemicals, sometimes present at concentrations or in combinations which can have a negative impact on human health. Indoor Air Quality (IAQ) surveys are often required to characterize human exposure or to investigate IAQ concerns and complaints. Such surveys should include sufficient contextual information to elucidate sources, pathways, and the magnitude of exposures. The aim of this review was to investigate and describe the parameters that affect IAQ in residential dwellings: building location, layout, and ventilation, finishing materials, occupant activities, and occupant demography. About 180 peer-reviewed articles, published from 01/2013 to 09/2021 (plus some important earlier publications), were reviewed. The importance of the building parameters largely depends on the study objectives and whether the focus is on a specific pollutant or to assess health risk. When considering classical pollutants such as particulate matter (PM) or volatile organic compounds (VOCs), the building parameters can have a significant impact on IAQ, and detailed information of these parameters needs to be reported in each study. Research gaps and suggestions for the future studies together with recommendation of where measurements should be done are also provided.


Assuntos
Poluentes Atmosféricos , Poluição do Ar em Ambientes Fechados , Poluentes Ambientais , Humanos , Poluição do Ar em Ambientes Fechados/análise , Poluentes Atmosféricos/análise , Monitoramento Ambiental , Material Particulado/análise
17.
Salud Publica Mex ; 64(3, may-jun): 328-332, 2022 Jun 02.
Artigo em Inglês | MEDLINE | ID: mdl-36130383

RESUMO

OBJECTIVE: To estimate Covid-19 and pre-pandemic low respiratory infection (LRI) mortality in children and adolescents in Mexico. MATERIALS AND METHODS: We estimated the percentage of total mortality attributable to Covid-19 (95% confidence intervals; 95%CI) and made the corresponding estimates for pre-pandemic LRI mortality. RESULTS: In 2019, LRIs represented 8.6% (95%CI 8.3, 8.9) of deaths in children aged 0-9 years, and 2.0% (95%CI 1.8, 2.3) in those aged 10-19 years. In 2020, the corresponding estimates for Covid-19 were 4.4% (95%CI 4.1, 4.6) and 3.7% (95%CI 3.4, 4.1). CONCLUSIONS: Relative to LRI, Covid-19 may be exerting a considerable mortality burden, particularly in older children and adolescents.


Assuntos
COVID-19 , Infecções Respiratórias , Adolescente , Criança , Humanos , México/epidemiologia
18.
Cancers (Basel) ; 14(14)2022 Jul 07.
Artigo em Inglês | MEDLINE | ID: mdl-35884384

RESUMO

The application to clinical practice of liquid biopsy in patients with lung cancer has led to an advance in the diagnosis and monitoring of the disease. Detection of alterations in EGFR genes related to TKI treatment in EGFR-mutated non-small cell lung cancer patients is a routine method in pathology laboratories. The primary objective of this work was to analyze the presence of EGFR mutations in cfDNA of 86 patients with lung cancer undergoing oncological treatment related to response to treatment with TKIs. Secondarily, we evaluated the dynamics of EGFR mutations, the presence of the T790M alteration and its relationship with drug resistance and analyzed by NGS molecular alterations in cfDNA of patients with discordant progression. Our results demonstrate that understanding the mutational status of patients treated with TKIs over time is essential to monitor disease progression. In this context, liquid biopsy is a fundamental key. In addition, it is not only necessary to detect EGFR mutations, but also other concomitant mutations that would be influencing the development of the disease. In this sense, we have discovered that mutations in the NF1 tumor suppressor gene could be exerting an as yet unknown function in lung cancer.

19.
Rev Assoc Med Bras (1992) ; 68(6): 808-813, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35766696

RESUMO

OBJECTIVE: The aim of this study was to describe the medical nutritional therapy (MNT) of adult non-critically ill hospitalization patients. METHODS: In a retrospective study, adults hospitalized for more than 48 h in non-intensive care unit medical and surgical areas that were classified as being at nutritional risk were included. Malnutrition was defined according to Global Leadership Initiative on Malnutrition (GLIM) criteria. RESULTS: A total of 255 patients, aged 54.13±18.4 years, who were at risk of malnutrition were included in this study. Of these, 50% were males. Notably, 52.5% received oral nutrition supplementation (ONS), 23.5% enteral nutrition (EN), 15% parenteral nutrition (PN), and 9% received enteral and parenteral nutrition (EPN). Patients with EPN presented the highest frequency of malnutrition (52%), and therefore they received more than 100% of energy and protein requirements. The median length of stay was 25 days. Among patients with nutritional risk receiving EPN, no deaths occurred. Patients, identified at nutritional risk, but without malnutrition according to GLIM, and receiving ONS had significantly lower mortality than patients receiving other MNT. CONCLUSIONS: Oral nutrition supplementation was the more frequent MNT prescribed. The frequency of malnutrition and percentage of prescribed energy and protein were higher in patients receiving PN and EPN compared with those receiving ONS.


Assuntos
Estado Terminal , Desnutrição , Adulto , Estado Terminal/terapia , Feminino , Humanos , Tempo de Internação , Masculino , Desnutrição/terapia , Estado Nutricional , Nutrição Parenteral , Prognóstico , Estudos Retrospectivos , Centros de Atenção Terciária
20.
Salud pública Méx ; 64(3): 328-332, May.-Jun. 2022. tab, graf
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1522944

RESUMO

Abstract: Objective: To estimate Covid-19 and pre-pandemic low respiratory infection (LRI) mortality in children and adolescents in Mexico. Materials and methods: We estimated the percentage of total mortality attributable to Covid-19 (95% confidence intervals; 95%CI) and made the corresponding estimates for pre-pandemic LRI mortality. Results: In 2019, LRIs represented 8.6% (95%CI 8.3, 8.9) of deaths in children aged 0-9 years, and 2.0% (95%CI 1.8, 2.3) in those aged 10-19 years. In 2020, the corresponding estimates for Covid-19 were 4.4% (95%CI 4.1, 4.6) and 3.7% (95%CI 3.4, 4.1). Conclusions: Relative to LRI, Covid-19 may be exerting a considerable mortality burden, particularly in older children and adolescents.


Resumen: Objetivo: Estimar la mortalidad por Covid-19 e infección respiratoria baja (IRB) pre-pandémica en niños y adolescentes en México. Material y métodos: Se estimó el porcentaje de mortalidad atribuible a Covid-19 (intervalos de confianza 95%; IC95%) y se realizaron las estimaciones correspondientes para IRB pre-pandémica. Resultados: En 2019, las IRB representaron 8.6% (IC95% 8.3, 8.9) de las muertes en niños de 0-9 años y 2.0% (IC95% 1.8, 2.3) en aquéllos entre 10-19 años. Los valores correspondientes en 2020 para Covid-19 fueron 4.4% (IC95% 4.1, 4.6) y 3.7% (IC95% 3.4, 4.1). Conclusiones: En comparación con IRB, Covid-19 puede estar ejerciendo una carga de mortalidad considerable, particularmente en niños mayores y adolescentes.

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