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1.
JAMA Netw Open ; 7(5): e2411905, 2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38758554

RESUMO

Importance: Linking prenatal drug exposures to both infant behavior and adult cognitive outcomes may improve early interventions. Objective: To assess whether neonatal physical, neurobehavioral, and infant cognitive measures mediate the association between prenatal cocaine exposure (PCE) and adult perceptual reasoning IQ. Design, Setting, and Participants: This study used data from a longitudinal, prospective birth cohort study with follow-up from 1994 to 2018 until offspring were 21 years post partum. A total of 384 (196 PCE and 188 not exposed to cocaine [NCE]) infants and mothers were screened for cocaine or polydrug use. Structural equation modeling was performed from June to November 2023. Exposures: Prenatal exposures to cocaine, alcohol, marijuana, and tobacco assessed through urine and meconium analyses and maternal self-report. Main Outcomes and Measures: Head circumference, neurobehavioral assessment, Bayley Scales of Infant Development, Fagan Test of Infant Intelligence score, Wechsler Perceptual Reasoning IQ, Home Observation for Measurement of the Environment (HOME) score, and blood lead level. Results: Among the 384 mothers in the study, the mean (SD) age at delivery was 27.7 (5.3) years (range, 18-41 years), 375 of 383 received public assistance (97.9%) and 336 were unmarried (87.5%). Birth head circumference (standardized estimate for specific path association, -0.05, SE = 0.02; P = .02) and 1-year Bayley Mental Development Index (MDI) (standardized estimate for total of the specific path association, -0.05, SE = 0.02; P = .03) mediated the association of PCE with Wechsler Perceptual Reasoning IQ, controlling for HOME score and other substance exposures. Abnormal results on the neurobehavioral assessment were associated with birth head circumference (ß = -0.20, SE = 0.08; P = .01). Bayley Psychomotor Index (ß = 0.39, SE = 0.05; P < .001) and Fagan Test of Infant Intelligence score (ß = 0.16, SE = 0.06; P = .01) at 6.5 months correlated with MDI at 12 months. Conclusions and Relevance: In this cohort study, a negative association of PCE with adult perceptual reasoning IQ was mediated by early physical and behavioral differences, after controlling for other drug and environmental factors. Development of infant behavioral assessments to identify sequelae of prenatal teratogens early in life may improve long-term outcomes and public health awareness.


Assuntos
Cocaína , Inteligência , Efeitos Tardios da Exposição Pré-Natal , Humanos , Feminino , Gravidez , Adulto , Inteligência/efeitos dos fármacos , Lactente , Cocaína/efeitos adversos , Estudos Prospectivos , Masculino , Adulto Jovem , Adolescente , Comportamento do Lactente/efeitos dos fármacos , Estudos Longitudinais , Recém-Nascido , Desenvolvimento Infantil/efeitos dos fármacos
2.
PLoS One ; 19(5): e0301520, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38758830

RESUMO

White matter (WM) changes occur throughout the lifespan at a different rate for each developmental period. We aggregated 10879 structural MRIs and 6186 diffusion-weighted MRIs from participants between 2 weeks to 100 years of age. Age-related changes in gray matter and WM partial volumes and microstructural WM properties, both brain-wide and on 29 reconstructed tracts, were investigated as a function of biological sex and hemisphere, when appropriate. We investigated the curve fit that would best explain age-related differences by fitting linear, cubic, quadratic, and exponential models to macro and microstructural WM properties. Following the first steep increase in WM volume during infancy and childhood, the rate of development slows down in adulthood and decreases with aging. Similarly, microstructural properties of WM, particularly fractional anisotropy (FA) and mean diffusivity (MD), follow independent rates of change across the lifespan. The overall increase in FA and decrease in MD are modulated by demographic factors, such as the participant's age, and show different hemispheric asymmetries in some association tracts reconstructed via probabilistic tractography. All changes in WM macro and microstructure seem to follow nonlinear trajectories, which also differ based on the considered metric. Exponential changes occurred for the WM volume and FA and MD values in the first five years of life. Collectively, these results provide novel insight into how changes in different metrics of WM occur when a lifespan approach is considered.


Assuntos
Substância Branca , Humanos , Substância Branca/diagnóstico por imagem , Adulto , Masculino , Feminino , Adolescente , Pessoa de Meia-Idade , Idoso , Adulto Jovem , Criança , Idoso de 80 Anos ou mais , Lactente , Pré-Escolar , Envelhecimento/fisiologia , Longevidade , Recém-Nascido , Imagem de Tensor de Difusão , Imagem de Difusão por Ressonância Magnética , Anisotropia , Encéfalo/diagnóstico por imagem , Encéfalo/crescimento & desenvolvimento , Substância Cinzenta/diagnóstico por imagem
4.
Int J Colorectal Dis ; 39(1): 73, 2024 May 18.
Artigo em Inglês | MEDLINE | ID: mdl-38760622

RESUMO

BACKGROUND: The incidence of inflammatory bowel disease (IBD) is rising worldwide, but epidemiological data on children and adolescents are lacking. Understanding the global burden of IBD among children and adolescents is essential for global standardization of methodology and treatment options. METHODS: This is a cross-sectional study based on aggregated data. We estimated the prevalence and incidence of IBD in children and adolescents between 1990 and 2019 according to the Global Burden of Disease Study 2019 (GBD 2019). Age-standardized rates (ASRs) and estimated annual percentage changes (EAPCs) were used to compare the burden and trends between different regions and countries. RESULTS: In 2019, there were 25,659 new cases and 88,829 prevalent cases of IBD among children and adolescents globally, representing an increase of 22.8% and 18.5%, respectively, compared to 1990. Over the past 30 years, the incidence and prevalence of IBD among children and adolescents have been highest in high SDI regions, with the most significant increases in East Asia and high-income Asia Pacific. At the age level, incidence and prevalence were significantly higher in the 15-19-year-old age group, while the < 5-year-old group showed the most significant increase in incidence and prevalence. CONCLUSION: The incidence of IBD in children and adolescents is significantly on the rise in some countries and regions, and IBD will remain an important public health issue with extensive healthcare and economic costs in the future. The reported IBD burden in children and adolescents at the global, regional, and national levels will assist in the development of more precise health policies.


Assuntos
Doenças Inflamatórias Intestinais , Humanos , Adolescente , Criança , Doenças Inflamatórias Intestinais/epidemiologia , Incidência , Prevalência , Pré-Escolar , Masculino , Feminino , Estudos Transversais , Adulto Jovem , Saúde Global , Lactente
5.
Pediatr Rheumatol Online J ; 22(1): 55, 2024 May 17.
Artigo em Inglês | MEDLINE | ID: mdl-38760816

RESUMO

BACKGROUND: Systemic autoinflammatory disorders (SAIDs) represent a growing spectrum of diseases characterized by dysregulation of the innate immune system. The most common pediatric autoinflammatory fever syndrome, Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA), has well defined clinical diagnostic criteria, but there is a subset of patients who do not meet these criteria and are classified as undefined autoinflammatory diseases (uAID). This project, endorsed by PRES, supported by the EMERGE fellowship program, aimed to analyze the evolution of symptoms in recurrent fevers without molecular diagnosis in the context of undifferentiated AIDs, focusing on PFAPA and syndrome of undifferentiated recurrent fever (SURF), using data from European AID registries. METHODS: Data of patients with PFAPA, SURF and uSAID were collected from 3 registries including detailed epidemiological, demographic and clinical data, results of the genetic testing and additional laboratory investigations with retrospective application of the modified Marshall and PRINTO/Eurofever classification criteria on the cohort of PFAPA patients and preliminary SURF criteria on uSAID/SURF patients. RESULTS: Clinical presentation of PFAPA is variable and some patients did not fit the conventional PFAPA criteria and exhibit different symptoms. Some patients did not meet the criteria for either PFAPA or SURF, highlighting the heterogeneity within these groups. The study also explored potential overlaps between PFAPA and SURF/uAID, revealing that some patients exhibited symptoms characteristic of both conditions, emphasizing the need for more precise classification criteria. CONCLUSIONS: Patients with recurrent fevers without molecular diagnoses represent a clinically heterogeneous group. Improved classification criteria are needed for both PFAPA and SURF/uAID to accurately identify and manage these patients, ultimately improving clinical outcomes.


Assuntos
Doenças Hereditárias Autoinflamatórias , Linfadenite , Faringite , Sistema de Registros , Estomatite Aftosa , Humanos , Criança , Europa (Continente)/epidemiologia , Feminino , Masculino , Estomatite Aftosa/diagnóstico , Estomatite Aftosa/epidemiologia , Pré-Escolar , Doenças Hereditárias Autoinflamatórias/diagnóstico , Linfadenite/diagnóstico , Linfadenite/epidemiologia , Faringite/diagnóstico , Adolescente , Lactente , Estudos Retrospectivos , Febre/etiologia , Febre/diagnóstico , Recidiva
6.
Dermatol Online J ; 30(1)2024 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-38762861

RESUMO

Juvenile xanthogranuloma is the most frequent form of non-Langerhans cell histiocytosis in children. Clinically, it presents as well defined, yellowish papules that are typically located on the head, neck, upper trunk, and proximal region of the extremities. Although solitary lesions are the most common presentation, few cases of multiple juvenile xanthogranuloma have been described, more frequently associated with extracutaneous involvement. We report a 2-month-old girl with 22 cutaneous papules, clinically and histologically compatible with juvenile xanthogranulomas. Screening of visceral involvement was performed with no evidence of systemic disease. Identifying high-risk factors of systemic disease in patients with multiple juvenile xanthogranuloma is essential to perform an appropriate management of this entity.


Assuntos
Xantogranuloma Juvenil , Humanos , Xantogranuloma Juvenil/patologia , Xantogranuloma Juvenil/diagnóstico , Feminino , Lactente
7.
J Health Econ ; 95: 102876, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38763530

RESUMO

Prior research has found that a high level of residential racial segregation, or the degree to which racial/ethnic groups are isolated from one another, is associated with worsened infant health outcomes, particularly among non-Hispanic (NH) Black infant populations. However, because exposure to segregation is non-random, it is unclear whether and to what extent segregation is causally linked to infant health. To overcome this empirical limitation, we leverage exogenous variation in the placement of railroad tracks in the 19th century to predict contemporary segregation, an approach first introduced by Ananat (2011). In alignment with prior literature, we find that residential segregation has statistically significant associations with negative birth outcomes among Black infant populations in the area. Using OLS methods underestimates the negative impacts of segregation on infant health. We fail to detect comparable effects on health outcomes among NH White infant populations. Further, we identify several key mechanisms by which residential segregation could influence health outcomes among Black infant populations, including lower access to prenatal care during the first trimester, higher levels of anti-Black prejudice, greater transportation barriers, and increased food insecurity. Given that poor birth outcomes have adverse effects on adults' health and well-being, the findings suggest that in-utero exposure to residential segregation could have important implications for Black-White inequality over the life course.


Assuntos
Saúde do Lactente , Segregação Social , Humanos , Feminino , Lactente , Negro ou Afro-Americano/estatística & dados numéricos , Recém-Nascido , Gravidez , Estados Unidos , Masculino , Características de Residência , Adulto , Disparidades nos Níveis de Saúde
8.
Food Res Int ; 187: 114304, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38763623

RESUMO

This study evaluated muti-mycotoxins in 199 samples including processed infant foods and raw materials collected randomly from an infant food company and assessed their role in dietary exposure in infants and young children via probabilistic risk assessment. Approximately 79.6 % (74/93) of the processed infant foods and 65.1 % (69/106) of the raw materials were contaminated by mycotoxins, with a mean occurrence level of 3.66-321.8 µg/kg. Deoxynivalenol (DON) and tenuazonic acid (TeA) were the more prevalent mycotoxins detected, based on their higher frequencies and levels across samples. Co-occurrence of more than two mycotoxins was detected in 61.3 % (57/93) of the processed infant foods and 53.8 % (57/106) of the raw materials. Wheat flour and derived products (e.g., infant noodles and infant biscuits) were contaminated with higher contamination levels and a greater variety of mycotoxins than other samples (e.g., infant cereal and rice grains). The estimated daily exposure to OTA, DON, ZEN, and TEN was lower than the corresponding reference health-based guidance values, indicating acceptable health risks. However, the estimated dietary exposure to alternariol monomethyl ether (AME), alternariol (AOH), and tenuazonic acid (TeA) exceeded the corresponding thresholds of toxicological concern values, indicating potential dietary intake risks. Among the various samples, cereals and cereal-based infant foods emerged as the primary contributors to mycotoxin exposure. Further research is advised to address the uncertainties surrounding the toxicity associated with emerging Alternaria mycotoxins and to conduct cumulative risk assessments concerning multiple mycotoxin exposure in infants and young children.


Assuntos
Exposição Dietética , Contaminação de Alimentos , Alimentos Infantis , Micotoxinas , Micotoxinas/análise , Medição de Risco , Alimentos Infantis/análise , Humanos , Contaminação de Alimentos/análise , Lactente , China , Exposição Dietética/análise , Exposição Dietética/efeitos adversos , Grão Comestível/química , Grão Comestível/microbiologia , Farinha/análise , Tricotecenos/análise , Microbiologia de Alimentos
9.
Food Res Int ; 187: 114389, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38763651

RESUMO

Ochratoxin A (OTA), zearalenone (ZEN), and deoxynivalenol (DON) are mycotoxins whose exposure is associated with various adverse health effects, including cancer and renal disorders, estrogenic effects, and immunosuppressive and gastrointestinal disorders, respectively. Infants (<2 years) are the most vulnerable group to mycotoxins, representing a unique combination of restricted food consumption types, low body weight, lower ability to eliminate toxins, and more future years to accumulate toxins. This study aimed to estimate the infant́s exposure to OTA, DON, and ZEN due to the consumption of milk formula and baby cereals in Chile. Milk formula samples (n = 41) and baby cereals (n = 30) were collected and analyzed using commercial ELISA kits for OTA, DON, and ZEA determination. Exposure was assessed by the Estimated Daily Intake (EDI) approach (mean and worst-case scenario, WCS) with the levels found in a modified Lower Bound (mLB) and Upper Bound (UB); ideal consumption (<6m, 7-12 m, and 13-24 m); adjusted by the weight of each group. The risk was estimated by comparing the EDI with a reference tolerable daily intake or by the margin of exposure (MOE) in the case of OTA. DON and OTA occurrence in infant formula were 34 % and 41 %, respectively. The co-occurrence between these mycotoxins was 22 %. Mycotoxin contents were below LOQ values except for OTA determined in one sample (0.29 ng/ml). No milk formulae were contaminated with ZEN. In the case of baby cereals, the occurrences were 17 % for OTA, 30 % for DON, and 7 % for ZEN, all below LOQ. Co-occurrence was seen in two samples between ZEN and OTA. According to exposure calculations, the MOE for OTA was less than 10,000 in all models for milk formula between 0 to 12 months of age and in the UB and WCS for cereal consumption. Health concerns were observed for DON in the WCS and UB for milk consumption in all ages and only in the UB WCS for cereal consumption. Considering the high consumption of milk formula in these age groups, regulation of OTA and other co-occurring mycotoxins in infant milk and food is strongly suggested.


Assuntos
Exposição Dietética , Grão Comestível , Contaminação de Alimentos , Fórmulas Infantis , Ocratoxinas , Tricotecenos , Zearalenona , Humanos , Zearalenona/análise , Fórmulas Infantis/química , Chile , Grão Comestível/química , Lactente , Tricotecenos/análise , Contaminação de Alimentos/análise , Ocratoxinas/análise , Exposição Dietética/análise , Exposição Dietética/efeitos adversos , Medição de Risco , Recém-Nascido , Alimentos Infantis/análise
10.
Clin Oral Investig ; 28(6): 326, 2024 May 20.
Artigo em Inglês | MEDLINE | ID: mdl-38763957

RESUMO

OBJECTIVES: To three-dimensionally assess differences in craniomaxillofacial skeletal development in patients with operated unilateral cleft lip and palate (UCLP) treated with/without presurgical nasoalveolar molding (PNAM) with a mean age of 5 years. MATERIALS AND METHODS: Cone-beam CT radiographs of 30 patients with UCLP who had undergone PNAM and 34 patients with UCLP who did not receive PNAM were analyzed. The data were stored in DICOM file format and were imported into the Dolphin Imaging program for 3D image reconstruction and landmark identification. 33 landmarks, 17 linear and three angular variables representing craniofacial morphology were analyzed and compared by using the Mann-Whitney U tests. RESULTS: The vast majority of linear variables and 3D coordinates of landmark points reflecting craniofacial skeletal symmetry were not significantly different between the two groups. In terms of craniofacial skeletal development, the PNAM group had a significantly smaller anterior nasal spine offset in the midsagittal plane and a greater maxillary length compared to the non-PNAM group. CONCLUSIONS: Evaluations performed in early childhood showed that treatment with/without PNAM in the neonatal period was not a major factor influencing craniomaxillofacial hard tissue development in patients with UCLP; moreover, PNAM treatment showed significant correction of skeletal deviation at the base of the nose. CLINICAL RELEVANCE: Follow-up in early childhood has shown that PNAM treatment administered during the neonatal stage does not impede maxillary development and has benefits in correcting nasal floor deviation. It is a viable option for improving nasal deformity in children with unilateral cleft lip and palate.


Assuntos
Fenda Labial , Fissura Palatina , Tomografia Computadorizada de Feixe Cônico , Imageamento Tridimensional , Moldagem Nasoalveolar , Humanos , Fenda Labial/terapia , Fenda Labial/diagnóstico por imagem , Fissura Palatina/terapia , Fissura Palatina/diagnóstico por imagem , Estudos Retrospectivos , Masculino , Feminino , Pré-Escolar , Imageamento Tridimensional/métodos , Resultado do Tratamento , Desenvolvimento Maxilofacial , Pontos de Referência Anatômicos , Lactente
11.
Eur Rev Med Pharmacol Sci ; 28(9): 3384-3390, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38766795

RESUMO

OBJECTIVE: We aimed to investigate the application of CD34 detection in immunophenotypic discrimination and its prognostic relevance in children with acute B-lymphoblastic leukemia (B-ALL). PATIENTS AND METHODS: A retrospective analysis was conducted on clinical follow-up data of 105 children with newly diagnosed B-ALL treated at our hospital from January 2022 to December 2023. Based on the expression of CD34 in the bone marrow, patients were divided into a CD34 positive group (positive cells ≥10%) and a CD34 negative group (positive cells <10%). The study compared the positive rates of common leukemia cell antigens, clinical characteristics, initial treatment responses, and long-term follow-up outcomes between the two groups. RESULTS: Among all 105 B-ALL cases, 87 children (82.9%) had bone marrow CD34 positive cells ≥10%, classified into the CD34 positive group, while the remaining 18 children (17.1%) had bone marrow CD34 positive cells <10%, classified into the CD34 negative group. The CD34 positive group exhibited significantly higher positive rates of CD13 expression, standard-risk B-ALL, and risk stratification than the CD34 negative group. In contrast, the proportions of early pre-B-ALL, E2A-PBX1 fusion gene, and MLL-AF4 fusion gene were significantly lower in the CD34 negative group, with statistically significant differences (p<0.05). No significant differences were found in the positive rates of leukemia cell antigens such as CD10, CD19, CD20, CD22, CD79a, CD13, CD33, and CD38 between the two groups (p>0.05). The occurrence rates of minimal residual disease (MRD) and relapse after induction chemotherapy in the CD34 positive group were significantly lower than those in the CD34 negative group (p<0.05). However, the sensitivity to the first prednisone treatment and bone marrow treatment efficacy on the 19th and 33rd days after chemotherapy showed no significant differences between the groups (p>0.05). CONCLUSIONS: A higher positive rate of bone marrow CD34 expression in children with B-ALL is associated with a favorable prognosis. Children with negative CD34 expression are relatively more prone to MRD and tumor relapse after chemotherapy.


Assuntos
Antígenos CD34 , Imunofenotipagem , Leucemia-Linfoma Linfoblástico de Células Precursoras B , Humanos , Criança , Antígenos CD34/metabolismo , Masculino , Feminino , Pré-Escolar , Estudos Retrospectivos , Prognóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras B/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras B/imunologia , Lactente , Adolescente
12.
Pediatr Transplant ; 28(4): e14784, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38766976

RESUMO

BACKGROUND: The goal of this study was to assess the effect of donor type and pre-transplant immunotherapy (IST) on outcomes of hematopoietic stem cell transplantation (HSCT) for children and young adults with severe aplastic anemia (SAA). METHODS: This retrospective, multi-center study included 52 SAA patients, treated in 5 pediatric transplant programs in Florida, who received HSCT between 2010 and 2020 as the first- or second-line treatment. RESULTS: The median age at HSCT for all 52 patients was 15 years (range 1-25). The 3-year overall survival (OS) by donor type were as follows: 95% [95% CI 85.4-99] for matched related donors (MRD) (N = 24), 84% [95% CI 63.5-99] for haploidentical (N = 13), and 71% [95% CI 36-99] for matched unrelated donors (MUD) (N = 7). The 3-year OS was 81% [95% CI 69.7-99] for all patients, 90.5% [95% CI 79.5-99] for non-IST patients (N = 27), and 70% [95% CI 51-99] for IST patients (N = 24) (log-rank p = .04). Survival of haploidentical HSCT (haplo-HSCT) recipients with post-transplant cyclophosphamide (PTCy) (N = 13) was excellent for both groups: 100% for non-IST patients (N = 3) and 80% for IST patients (N = 10). The 3-year OS for patients with previous IST by donor type in groups where >5 patients were available was 78.8% [95% CI 52.3-99] for haplo-HSCT (N = 10) and 66.7% [95% CI 28.7-99] for MUD (N = 6). Although it appears that patients receiving HSCT ≥6 months after the start of IST had worse survival, the number of patients in each category was small and log-rank was not significant(p = .65). CONCLUSIONS: Patients receiving MUD and haplo-HSCT with PTCy had similar outcomes, suggesting that haplo-HSCT with PTCy could be included in randomized trials of upfront IST versus alternative donor HSCT.


Assuntos
Anemia Aplástica , Transplante de Células-Tronco Hematopoéticas , Humanos , Anemia Aplástica/terapia , Adolescente , Criança , Estudos Retrospectivos , Masculino , Feminino , Pré-Escolar , Adulto Jovem , Adulto , Lactente , Resultado do Tratamento , Terapia de Imunossupressão/métodos , Doadores de Tecidos , Imunossupressores/uso terapêutico
13.
Pediatr Transplant ; 28(4): e14787, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38766980

RESUMO

BACKGROUND: Children awaiting heart transplant (Tx) have a high risk of death due to donor organ scarcity. Historically, ventricular assist devices (VADs) reduced waitlist mortality, prompting increased VAD use. We sought to determine whether the VAD survival benefit persists in the current era. METHODS: Using the Scientific Registry of Transplant Recipients, we identified patients listed for Tx between 3/22/2016 and 9/1/2020. We compared characteristics of VAD and non-VAD groups at Tx listing. Cox proportional hazards models were used to identify risk factors for 1-year waitlist mortality. RESULTS: Among 5054 patients, 764 (15%) had a VAD at Tx listing. The VAD group was older with more mechanical ventilation and renal impairment. Unadjusted waitlist mortality was similar between groups; the curves crossed ~90 days after listing (p = .55). In multivariable analysis, infant age (HR 2.77, 95%CI 2.13-3.60), Black race (HR 1.57, 95%CI 1.31-1.88), congenital heart disease (HR 1.23, 95%CI 1.04-1.46), renal impairment (HR 2.67, 95%CI 2.19-3.26), inotropes (HR 1.28, 95%CI 1.09-1.52), and mechanical ventilation (HR 2.23, 95%CI 1.84-2.70) were associated with 1-year waitlist mortality. VADs were not associated with mortality in the first 90 waitlist days but were protective for those waiting ≥90 days (HR 0.43, 95%CI 0.26-0.71). CONCLUSIONS: In the current era, VADs reduce waitlist mortality, but only for those waitlisted ≥90 days. The differential effect by race, size, and VAD type is less clear. These findings suggest that Tx listing without VAD may be reasonable if a short waitlist time is anticipated, but VADs may benefit those expected to wait >90 days.


Assuntos
Transplante de Coração , Coração Auxiliar , Sistema de Registros , Listas de Espera , Humanos , Listas de Espera/mortalidade , Masculino , Feminino , Lactente , Criança , Pré-Escolar , Adolescente , Fatores de Risco , Bases de Dados Factuais , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/cirurgia , Insuficiência Cardíaca/terapia , Estados Unidos/epidemiologia
14.
Pediatr Transplant ; 28(4): e14780, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38766999

RESUMO

BACKGROUND: The aims of this study are to report our experience with treosulfan-based conditioning regimens for patients with non-malignant hematologic conditions, correlating clinical outcomes at different time points post-transplant with treosulfan exposure (AUC). METHODS: This study was a single-center observational study investigating overall survival (OS), disease-free survival (DFS), and event-free survival (EFS) end-points post-transplant. The consequences of treosulfan AUC with respect to toxicity, correction of underlying disease, and long-term chimerism were also explored using pharmacokinetic analysis. RESULTS: Forty-six patients received 49 transplants with treosulfan and fludarabine-based conditioning between 2005 and 2023. Twenty-four patients also received thiotepa. Donor chimerism was assessed on either whole blood or sorted cell lines at different time points post-transplant. Thirty-nine patients received treosulfan pharmacokinetic assessment to evaluate cumulative AUC, with five infants receiving real-time assessment to facilitate daily dose adjustment. OS, DFS, and EFS were 87%, 81%, and 69%, respectively. Median follow-up was 32.1 months (range 0.82-160 months) following transplant. Lower EFS was associated with patient age (<1 year; p = .057) and lower cumulative treosulfan dose (<42 g/m2; p = .003). Stable donor chimerism in B-cell, NK-cell, and granulocyte lineages at 1-year post-transplant were more prevalent in patients receiving thiotepa conditioning. Two infants required daily dose adjustment to treosulfan to avoid high AUC. CONCLUSIONS: Excellent clinical outcomes and stable chimerism were observed in this patient series. The addition of thiotepa conferred no significant toxicity and trended toward sustained ongoing donor engraftment. Correlating treosulfan AUC with long-term patient outcomes is required.


Assuntos
Bussulfano , Transplante de Células-Tronco Hematopoéticas , Condicionamento Pré-Transplante , Humanos , Bussulfano/análogos & derivados , Bussulfano/uso terapêutico , Bussulfano/farmacocinética , Bussulfano/administração & dosagem , Condicionamento Pré-Transplante/métodos , Masculino , Transplante de Células-Tronco Hematopoéticas/métodos , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Adulto , Resultado do Tratamento , Estudos Retrospectivos , Vidarabina/análogos & derivados , Vidarabina/uso terapêutico , Vidarabina/administração & dosagem , Tiotepa/uso terapêutico , Tiotepa/administração & dosagem , Tiotepa/farmacocinética , Intervalo Livre de Doença , Seguimentos , Doenças Hematológicas/terapia , Antineoplásicos Alquilantes/uso terapêutico , Antineoplásicos Alquilantes/farmacocinética , Antineoplásicos Alquilantes/administração & dosagem
15.
Pediatr Transplant ; 28(4): e14783, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38767019

RESUMO

BACKGROUND: We hypothesized that alemtuzumab use is safe in pediatric kidney transplant recipients (KTRs) with equivalent long-term outcomes compared to other induction agents. METHODS: Using pediatric kidney transplant recipient data in the UNOS database between January 1, 2000, and June 30, 2022, multivariate logistic regression, multivariable Cox regression, and survival analyses were utilized to estimate the likelihoods of 1st-year and all-time hospitalizations, acute rejection, CMV infection, delayed graft function (DGF), graft loss, and patient mortality among recipients of three common induction regimens (ATG, alemtuzumab, and basiliximab). RESULTS: There were no differences in acute rejection or graft failure among induction or maintenance regimens. Basiliximab was associated with lower odds of DGF in deceased donor recipients (OR 0.77 [0.60-0.99], p = .04). Mortality was increased in patients treated with steroid-containing maintenance (HR 1.3 [1.005-1.7] p = .045). Alemtuzumab induction correlated with less risk of CMV infection than ATG (OR 0.76 [0.59-0.99], p = .039). Steroid-containing maintenance conferred lower rate of PTLD compared to steroid-free maintenance (HR 0.59 [0.4-0.8] p = .001). Alemtuzumab was associated with less risk of hospitalization within 1 year (OR 0.79 [0.67-0.95] p = .012) and 5 years (HR 0.54 [0.46-0.65] p < .001) of transplantation. Steroid maintenance also decreased 5 years hospitalization risk (HR 0.78 [0.69-0.89] p < .001). CONCLUSIONS: Pediatric KTRs may be safely treated with alemtuzumab induction without increased risk of acute rejection, DGF, graft loss, or patient mortality. The decreased risk of CMV infections and lower hospitalization rates compared to other agents make alemtuzumab an attractive choice for induction in pediatric KTRs, especially in those who cannot tolerate ATG.


Assuntos
Alemtuzumab , Basiliximab , Rejeição de Enxerto , Hospitalização , Imunossupressores , Transplante de Rim , Humanos , Alemtuzumab/uso terapêutico , Criança , Masculino , Hospitalização/estatística & dados numéricos , Feminino , Rejeição de Enxerto/prevenção & controle , Imunossupressores/uso terapêutico , Adolescente , Pré-Escolar , Basiliximab/uso terapêutico , Lactente , Sobrevivência de Enxerto , Soro Antilinfocitário/uso terapêutico , Resultado do Tratamento , Estudos Retrospectivos , Função Retardada do Enxerto/epidemiologia , Infecções por Citomegalovirus
16.
J Med Virol ; 96(5): e29679, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38767190

RESUMO

Acute gastroenteritis (AGE) represents a world public health relevant problem especially in children. Enteric viruses are the pathogens mainly involved in the episodes of AGE, causing about 70.00% of the cases. Apart from well-known rotavirus (RVA), adenovirus (AdV) and norovirus (NoV), there are various emerging viral pathogens potentially associated with AGE episodes. In this study, the presence of ten different enteric viruses was investigated in 152 fecal samples collected from children hospitalized for gastroenteritis. Real time PCR results showed that 49.3% of them were positive for viral detection with the following prevalence: norovirus GII 19.7%, AdV 15.8%, RVA 10.5%, human parechovirus (HPeV) 5.3%, enterovirus (EV) 3.3%, sapovirus (SaV) 2.6%. Salivirus (SalV), norovirus GI and astrovirus (AstV) 1.3% each, aichivirus (AiV) found in only one patient. In 38.2% of feces only one virus was detected, while co-infections were identified in 11.8% of the cases. Among young patients, 105 were ≤5 years old and 56.0% tested positive for viral detection, while 47 were >5 years old with 40.0% of them infected. Results obtained confirm a complex plethora of viruses potentially implicated in gastroenteritis in children, with some of them previously known for other etiologies but detectable in fecal samples. Subsequent studies should investigate the role of these viruses in causing gastroenteritis and explore the possibility that other symptoms may be ascribed to multiple infections.


Assuntos
COVID-19 , Coinfecção , Fezes , Gastroenterite , Humanos , Gastroenterite/virologia , Gastroenterite/epidemiologia , Pré-Escolar , Coinfecção/virologia , Coinfecção/epidemiologia , Fezes/virologia , Lactente , Itália/epidemiologia , Criança , Masculino , Feminino , COVID-19/epidemiologia , COVID-19/virologia , Sapovirus/isolamento & purificação , Sapovirus/genética , Vírus/isolamento & purificação , Vírus/classificação , Vírus/genética , Prevalência , Norovirus/isolamento & purificação , Norovirus/genética , Adolescente , Viroses/epidemiologia , Viroses/virologia , Recém-Nascido , SARS-CoV-2 , Rotavirus/isolamento & purificação , Rotavirus/genética , Adenoviridae/isolamento & purificação
17.
Pediatr Surg Int ; 40(1): 135, 2024 May 20.
Artigo em Inglês | MEDLINE | ID: mdl-38767779

RESUMO

AIM: Van der Zee (VdZ) described a technique to elongate the oesophagus in long-gap oesophageal atresia (LGOA) by thoracoscopic placement of external traction sutures (TPETS). Here, we describe our experience of using this technique. METHOD: Retrospective review of all LGOA + / - distal tracheo-oesophageal fistula (dTOF) cases where TPETS was used in our institutions. Data are given as medians (IQR). RESULTS: From 01/05/2019 to 01/03/2023, ten LGOA patients were treated by the VdZ technique. Five had oesophageal atresia (Gross type A or B, Group 1) and five had OA with a dTOF (type C, Group 2) but with a long gap precluding primary anastomosis. Age of first traction procedure was Group 1 = 53 (29-55) days and Group 2 = 3 (1-49) days. Median number of traction procedures = 3; time between first procedure and final anastomosis was 6 days (4-7). Four cases were converted to thoracotomy at the third procedure. Three had anastomotic leaks managed conservatively. Follow-up was 12-52 months. All patients achieved oesophageal continuity and were orally fed; no patient required an oesophagostomy. CONCLUSION: In this series, TPETS in LGOA facilitated delayed primary anastomoses and replicated the good results previously described but, in addition, was successful in cases with dTOF. We believe traction suture placement and tensioning benefit from being performed thoracoscopically because of excellent visualisation and the fact that the tension does not change when the chest is closed. Surgical and anaesthetic planning and expertise are crucial. It is now our management of choice in OA patients with a long gap with or without a distal TOF.


Assuntos
Atresia Esofágica , Técnicas de Sutura , Toracoscopia , Humanos , Atresia Esofágica/cirurgia , Estudos Retrospectivos , Toracoscopia/métodos , Masculino , Feminino , Recém-Nascido , Lactente , Fístula Traqueoesofágica/cirurgia , Tração/métodos , Resultado do Tratamento , Anastomose Cirúrgica/métodos , Esôfago/cirurgia , Esôfago/anormalidades
19.
BMC Pediatr ; 24(1): 347, 2024 May 20.
Artigo em Inglês | MEDLINE | ID: mdl-38769496

RESUMO

BACKGROUND: Among the neurological complications of influenza in children, the most severe is acute necrotizing encephalopathy (ANE), with a high mortality rate and neurological sequelae. ANE is characterized by rapid progression to death within 1-2 days from onset. However, the knowledge about the early diagnosis of ANE is limited, which is often misdiagnosed as simple seizures/convulsions or mild acute influenza-associated encephalopathy (IAE). OBJECTIVE: To develop and validate an early prediction model to discriminate the ANE from two common neurological complications, seizures/convulsions and mild IAE in children with influenza. METHODS: This retrospective case-control study included patients with ANE (median age 3.8 (2.3,5.4) years), seizures/convulsions alone (median age 2.6 (1.7,4.3) years), or mild IAE (median age 2.8 (1.5,6.1) years) at a tertiary pediatric medical center in China between November 2012 to January 2020. The random forest algorithm was used to screen the characteristics and construct a prediction model. RESULTS: Of the 433 patients, 278 (64.2%) had seizures/convulsions alone, 106 (24.5%) had mild IAE, and 49 (11.3%) had ANE. The discrimination performance of the model was satisfactory, with an accuracy above 0.80 from both model development (84.2%) and internal validation (88.2%). Seizures/convulsions were less likely to be wrongly classified (3.7%, 2/54), but mild IAE (22.7%, 5/22) was prone to be misdiagnosed as seizures/convulsions, and a small proportion (4.5%, 1/22) of them was prone to be misdiagnosed as ANE. Of the children with ANE, 22.2% (2/9) were misdiagnosed as mild IAE, and none were misdiagnosed as seizures/convulsions. CONCLUSION: This model can distinguish the ANE from seizures/convulsions with high accuracy and from mild IAE close to 80% accuracy, providing valuable information for the early management of children with influenza.


Assuntos
Influenza Humana , Convulsões , Humanos , Influenza Humana/complicações , Influenza Humana/diagnóstico , Pré-Escolar , Estudos Retrospectivos , Feminino , Masculino , Estudos de Casos e Controles , Convulsões/diagnóstico , Convulsões/etiologia , Criança , Lactente , Diagnóstico Diferencial , China/epidemiologia , Encefalopatias/diagnóstico , Encefalopatias/etiologia , Algoritmo Florestas Aleatórias
20.
BMC Cardiovasc Disord ; 24(1): 260, 2024 May 20.
Artigo em Inglês | MEDLINE | ID: mdl-38769516

RESUMO

INTRODUCTION: Use of doxorubicin, an anthracycline chemotherapeutic agent has been associated with late-occurring cardiac toxicities. Detection of early-occurring cardiac effects of cancer chemotherapy is essential to prevent occurrence of adverse events including toxicity, myocardial dysfunction, and death. OBJECTIVE: To investigate the prevalence of elevated cardiac troponin T (cTnT) and associated factors of myocardial injury in children on doxorubicin cancer chemotherapy. METHODS: Design: A cross-sectional study. SETTING AND SUBJECTS: A hospital-based study conducted on children aged 1-month to 12.4-years who had a diagnosis of cancer and were admitted at Kenyatta National Hospital (KNH). INTERVENTIONS AND OUTCOMES: The patients underwent Echocardiography (ECHO) before their scheduled chemotherapy infusion. Twenty-four (24) hours after the chemotherapy infusion the patients had an evaluation of the serum cardiac troponin T (cTnT) and a repeat ECHO. Myocardial injury was defined as cTnT level > 0.014 ng/ml or a Fractional Shortening (FS) of < 29% on ECHO. RESULTS: One hundred (100) children were included in the final analysis. Thirty-two percent (32%) of the study population had an elevated cTnT. A cumulative doxorubicin dose of > 175 mg/m2 was significantly associated with and elevated cTnT (OR, 10.76; 95% CI, 1.18-97.92; p = 0.035). Diagnosis of nephroblastoma was also associated with an elevated cTnT (OR, 3.0; 95% CI, 1.23-7.26) but not statistically significant (p = 0.105). Nine percent (9%) of the participants had echocardiographic evidence of myocardial injury. CONCLUSION: When compared to echocardiography, elevated levels of cTnT showed a higher association with early-occurring chemotherapy-induced myocardial injury among children on cancer treatment at a tertiary teaching and referral hospital in Kenya.


Assuntos
Antibióticos Antineoplásicos , Biomarcadores , Cardiotoxicidade , Doxorrubicina , Neoplasias , Centros de Atenção Terciária , Troponina T , Humanos , Estudos Transversais , Masculino , Feminino , Doxorrubicina/efeitos adversos , Criança , Quênia/epidemiologia , Troponina T/sangue , Pré-Escolar , Antibióticos Antineoplásicos/efeitos adversos , Lactente , Neoplasias/tratamento farmacológico , Neoplasias/sangue , Fatores de Risco , Biomarcadores/sangue , Prevalência , Fatores de Tempo , Regulação para Cima , Cardiopatias/induzido quimicamente , Cardiopatias/epidemiologia , Cardiopatias/diagnóstico por imagem , Cardiopatias/diagnóstico , Cardiopatias/sangue , Fatores Etários , Medição de Risco , Ecocardiografia
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