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1.
Am Heart J ; 271: 112-122, 2024 05.
Artículo en Inglés | MEDLINE | ID: mdl-38395293

RESUMEN

BACKGROUND: To date, there has been no independent core lab angiographic analysis of patients with COVID-19 and STEMI. The study characterized the angiographic parameters of patients with COVID-19 and STEMI. METHODS: Angiograms of patients with COVID-19 and STEMI from the North American COVID-19 Myocardial Infarction (NACMI) Registry were sent to a Core Laboratory in Vancouver, Canada. Culprit lesion(s), Thrombolysis In Myocardial Infarction (TIMI) flow, Thrombus Grade Burden (TGB), and percutaneous coronary intervention (PCI) outcome were assessed. RESULTS: From 234 patients, 74% had one culprit lesion, 14% had multiple culprits and 12% had no culprit identified. Multivessel thrombotic disease and multivessel CAD were found in 27% and 53% of patients, respectively. Stent thrombosis accounted for 12% of the presentations and occurred in 55% of patients with previous coronary stents. Of the 182 who underwent PCI, 60 (33%) had unsuccessful PCI due to post-PCI TIMI flow <3 (43/60), residual high thrombus burden (41/60) and/or thrombus related complications (27/60). In-hospital mortality for successful, partially successful, and unsuccessful PCI was 14%, 13%, and 27%, respectively. Unsuccessful PCI was associated with increased risk of in-hospital mortality (risk ratio [RR] 1.96; 95% CI: 1.05-3.66, P = .03); in the adjusted model this estimate was attenuated (RR: 1.24; 95% CI: 0.65-2.34, P = .51). CONCLUSION: In patients with COVID-19 and STEMI, thrombus burden was pervasive with notable rates of multivessel thrombotic disease and stent thrombosis. Post-PCI, persistent thrombus and sub-optimal TIMI 3 flow rates led to one-third of the PCI's being unsuccessful, which decreased over time but remained an important predictor of in-hospital mortality.


Asunto(s)
COVID-19 , Angiografía Coronaria , Intervención Coronaria Percutánea , Sistema de Registros , Infarto del Miocardio con Elevación del ST , Humanos , Infarto del Miocardio con Elevación del ST/terapia , Infarto del Miocardio con Elevación del ST/diagnóstico por imagen , COVID-19/complicaciones , COVID-19/terapia , Masculino , Femenino , Intervención Coronaria Percutánea/métodos , Intervención Coronaria Percutánea/estadística & datos numéricos , Persona de Mediana Edad , Anciano , Mortalidad Hospitalaria , SARS-CoV-2 , Trombosis Coronaria/diagnóstico por imagen , Canadá/epidemiología
2.
J Mol Recognit ; : e3089, 2024 Jun 18.
Artículo en Inglés | MEDLINE | ID: mdl-38894531

RESUMEN

The frequent use of anti-inflammatory drugs and the side effects of existing drugs keep the need for new compounds constant. For this purpose, flurbiprofen and ibuprofen-like compounds, which are frequently used anti-inflammatory compounds in this study, were synthesized and their structures were elucidated. Like ibuprofen and flurbiprofen, the compounds contain a residue of phenylacetic acid. On the other hand, it contains a secondary amine residue. Thus, it is planned to reduce the acidity, which is the biggest side effect of NSAI drugs, even a little bit. The estimated ADME parameters of the compounds were evaluated. Apart from internal use, local use of anti-inflammatory compounds is also very important. For this reason, the skin permeability values of the compounds were also calculated. And it has been found to be compatible with reference drugs. The COX enzyme inhibitory effects of the obtained compounds were tested by in vitro experiments. Compound 2a showed significant activity against COX-1 enzyme with an IC50 = 0.123 + 0.005 µM. The interaction of the compound with the enzyme active site was clarified by molecular dynamics studies.

3.
Cytotherapy ; 26(6): 556-566, 2024 06.
Artículo en Inglés | MEDLINE | ID: mdl-38483359

RESUMEN

BACKGROUND AIMS: Few human induced pluripotent stem cell (hiPSC) lines are Good Manufacturing Practice (GMP)-compliant, limiting the clinical use of hiPSC-derived products. Here, we addressed this by establishing and validating an in-house platform to produce GMP-compliant hiPSCs that would be appropriate for producing both allogeneic and autologous hiPSC-derived products. METHODS: Our standard research protocol for hiPSCs production was adapted and translated into a GMP-compliant platform. In addition to the generation of GMP-compliant hiPSC, the platform entails the methodology for donor recruitment, consent and screening, donor material procurement, hiPSCs manufacture, in-process control, specific QC test validation, QC testing, product release, hiPSCs storage and stability testing. For platform validation, one test run and three production runs were performed. Highest-quality lines were selected to establish master cell banks (MCBs). RESULTS: Two MCBs were successfully released under GMP conditions. They demonstrated safety (sterility, negative mycoplasma, endotoxins <5.0 EU/mL and negative adventitious agents), cell identity (>75% of cells expressing markers of undifferentiated state, identical STR profile, normal karyotype in >20 metaphases), purity (negative residual vectors and no plasmid integration in the genome) and potency (expression of at least two of the three markers for each of the three germ layers). In addition, directed differentiation to somitoids (skeletal muscle precursors) and six potential clinical products from all three germ layers was achieved: pancreatic islets (endoderm), kidney organoids and cardiomyocytes (mesoderm), and keratinocytes, GABAergic interneurons and inner-ear organoids (ectoderm). CONCLUSIONS: We successfully developed and validated a platform for generating GMP-compliant hiPSC lines. The two MCBs released were shown to differentiate into clinical products relevant for our own and other regenerative medicine interests.


Asunto(s)
Diferenciación Celular , Células Madre Pluripotentes Inducidas , Humanos , Células Madre Pluripotentes Inducidas/citología , Técnicas de Cultivo de Célula/métodos , Línea Celular
4.
Clin Exp Rheumatol ; 42(1): 194-201, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-37497697

RESUMEN

OBJECTIVES: Biological drugs are one of the most effective treatment methods for systemic juvenile idiopathic arthritis (SJIA) and can significantly prevent morbidity and mortality. This study aimed to evaluate the efficacy and safety of biologics in patients with SJIA and provide real-life data that might help improve the outcomes. METHODS: TURSIS was a retrospective multicentre study carried out in patients with SJIA for whom a biological treatment had been initiated between 1st March 2013 and 30th December 2018. Data include patients' characteristics, laboratory-clinical results, outcomes, and safety-related variables. The 24-month follow-up data of the patients and the efficacy and safety of biological drugs were evaluated. RESULTS: 147 patients were enrolled. The clinical course of the disease was as follows; it was monocyclic in 38.1%, polycyclic in 49%, and persistent in 12.9% of patients. First-choice biologics were interleukin (IL)-1 blockers in the majority of patients (56.5%), followed by the anti-IL-6 (25.2%) and anti-TNF-alpha drugs (18.4%). Anakinra was the most preferred biologic agent in patients with macrophage activation syndrome (MAS), and tocilizumab was used more frequently in patients with persistent type (p=0.000 and p=0.003). The most frequent switch rate was seen in patients receiving anakinra (n=40/68, 58.8%), and it was most frequently switched to canakinumab (n=32/40, 80%). Better physician's global assessment scores were achieved in patients treated with anakinra in Month 3, compared to other treatments (p=0.04). CONCLUSIONS: The results of our study support the efficacy of biological drugs in particular anti-IL-1 and anti-IL-6 drugs, in the treatment of SJIA. These treatments resulted in improvement in activity of disease and provide a considerable decrease in the frequency of MAS.


Asunto(s)
Artritis Juvenil , Productos Biológicos , Síndrome de Activación Macrofágica , Humanos , Artritis Juvenil/diagnóstico , Artritis Juvenil/tratamiento farmacológico , Proteína Antagonista del Receptor de Interleucina 1/efectos adversos , Turquía , Inhibidores del Factor de Necrosis Tumoral/uso terapéutico , Interleucina-1 , Productos Biológicos/efectos adversos , Síndrome de Activación Macrofágica/inducido químicamente
5.
Eur J Pediatr ; 183(7): 2937-2944, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38619568

RESUMEN

While most countries provide safe and effective influenza vaccines for at-risk groups, influenza vaccine coverage among children with rheumatic diseases remains uncertain. This study investigated influenza vaccination rates in children with juvenile idiopathic arthritis (JIA) during the 2019-2020 season and assessed the knowledge and attitudes of caregivers of children with JIA regarding influenza vaccination. The secondary aims were to identify barriers to vaccination and explore strategies to improve vaccination rates. A multi-centre, cross-sectional anonymous survey was conducted in 7 countries during the 2019-2020 influenza season to assess the uptake history of influenza vaccination. Among 287 participants, only 87 (30%) children with JIA received the influenza vaccine during the 2019-2020 season. Children who were more likely to be vaccinated were those with systemic juvenile idiopathic arthritis (sJIA), a history of previous vaccination and those aware of the vaccination recommendations. Conversely, children who previously experienced adverse vaccine-related events reported the lowest uptake. The primary reason for non-vaccination was lack of awareness about the necessity of influenza vaccination.  Conclusion: Despite variations among countries, the uptake of influenza vaccines remains low in children with JIA. Improving awareness among families about the importance of influenza vaccination may increase vaccination rates in children with rheumatic diseases. What is Known: • Rheumatic children are at increased risk for influenza infection due to immunosuppressive therapy and immune dysregulation. • Influenza vaccine is formally recommended to children with rheumatic diseases. What is New: • This multicentre study showed that influenza vaccine uptake rates remain suboptimal among children with Juvenile Idiopathic Arthritis despite formal recommendations. • Factors like previous experience with vaccination and information provided by medical professionals via different ways play essential roles in increasing vaccination rates and can contribute to improved health outcomes for these vulnerable children.


Asunto(s)
Artritis Juvenil , Vacunas contra la Influenza , Gripe Humana , Humanos , Estudios Transversales , Vacunas contra la Influenza/administración & dosificación , Masculino , Femenino , Niño , Gripe Humana/prevención & control , Preescolar , Conocimientos, Actitudes y Práctica en Salud , Adolescente , Vacunación/estadística & datos numéricos , Cobertura de Vacunación/estadística & datos numéricos
6.
Rheumatol Int ; 2024 Jul 16.
Artículo en Inglés | MEDLINE | ID: mdl-39012357

RESUMEN

Artificial intelligence algorithms, with roots extending into the past but experiencing a resurgence and evolution in recent years due to their superiority over traditional methods and contributions to human capabilities, have begun to make their presence felt in the field of pediatric rheumatology. In the ever-evolving realm of pediatric rheumatology, there have been incremental advancements supported by artificial intelligence in understanding and stratifying diseases, developing biomarkers, refining visual analyses, and facilitating individualized treatment approaches. However, like in many other domains, these strides have yet to gain clinical applicability and validation, and ethical issues remain unresolved. Furthermore, mastering different and novel terminologies appears challenging for clinicians. This review aims to provide a comprehensive overview of the current literature, categorizing algorithms and their applications, thus offering a fresh perspective on the nascent relationship between pediatric rheumatology and artificial intelligence, highlighting both its advancements and constraints.

7.
Pediatr Cardiol ; 45(5): 1007-1014, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38546846

RESUMEN

Early detection of cardiac involvement in Juvenile Dermatomyositis (JDM) is difficult due to the absence of clinical signs and symptoms, with systolic dysfunction often emerging in late stages and associated with a poor prognosis. This study aimed to employ two-dimensional speckle-tracking echocardiography (STE) for subclinical assessment of left ventricular (LV) systolic failure in JDM and explore potential associations between impaired LV systolic function (LV-GLS) and disease activity. A prospective study enrolled 20 healthy volunteers and 26 JDM patients (< 18 years old) without cardiac symptoms. Clinical data were collected from medical records, and echocardiograms were conducted by a pediatric cardiologist. Our study cohort demonstrated similar age to controls (13.5 ± .6 vs. 13.8 ± 4.7; p = 0.465). Median illness duration at echocardiography was 5 (1.5-17.5) years, and conventional echocardiography indicated normal LV ejection fraction (> 55%) in all participants. However, STE revealed lowered LV GLS in JDM patients (- 22.2 ± 4.1% vs. - 26.5 ± 5.3% p = 0.022). Pulse steroid users displayed lower GLS average values compared to non-users (ß = 4.99, 95% CI 1.34-8.64, p = 0.009). Negative correlations existed between LV-GLS and age at diagnosis (r = - 0.499; p = 0.011), diastolic parameters (E/E' ratio) and age at diagnosis (r = - 0.469; p = 0.018), as well as RV global strain and age at diagnosis (r = - 0.443; p = 0.024). Employing STE in JDM patients facilitated the identification of preclinical cardiac dysfunction. Given JDM patients' younger age, early myocardial damage detection through STE may impact treatment decisions and long-term cardiovascular prognosis.


Asunto(s)
Dermatomiositis , Ecocardiografía , Disfunción Ventricular Izquierda , Humanos , Dermatomiositis/complicaciones , Dermatomiositis/diagnóstico por imagen , Dermatomiositis/fisiopatología , Masculino , Femenino , Adolescente , Estudios Prospectivos , Ecocardiografía/métodos , Niño , Disfunción Ventricular Izquierda/diagnóstico por imagen , Disfunción Ventricular Izquierda/etiología , Disfunción Ventricular Izquierda/fisiopatología , Estudios de Casos y Controles , Volumen Sistólico , Sístole
8.
Clin Oral Investig ; 28(7): 392, 2024 Jun 22.
Artículo en Inglés | MEDLINE | ID: mdl-38907052

RESUMEN

OBJECTIVES: To investigate the four-year clinical outcome and marginal bone loss around narrow-diameter implants in patients with uncontrolled diabetes mellitus type 2 (T2DM) and normo-glycemic individuals. MATERIALS AND METHODS: In 11 T2DM patients with a concentration of glycated hemoglobin (HbA1C) > 6.5% (test group) and 15 normoglycemic patients (HbA1C < 6.0%; control group), one narrow-diameter tissue level implant, placed in the posterior maxilla or mandible, was investigated. The clinical parameters probing depth (PD), bleeding on probing (BOP), attachment loss (CAL), recession, and papilla bleeding index (PBI) were assessed manually after 24 and 48 months of function. The paired digital periapical radiographs were analyzed regarding the change in marginal bone level (MBL) from baseline to 48 months post-op. The technical complications were recorded. RESULTS: In the T2DM group, 11 patients were available for follow-ups. The overall implant survival rate after 48 months was 100%. The differences in means for the clinical parameters and the MBL between the T2DM and normo-glycemic patients for the observation period were statistically non-significant. No technical complications were recorded. CONCLUSIONS: The study demonstrated an encouraging clinical outcome with ND implants in patients with uncontrolled T2DM compared to non-diabetics after 48 months' post loading. CLINICAL RELEVANCE: Patients with HbA1C > 6.5% may benefit from the treatment with narrow-diameter implants by avoiding complex surgical interventions with augmentation procedures. REGISTRATION NUMBER (CLINICALTRIALS.GOV): NCT04630691.


Asunto(s)
Diseño de Prótesis Dental , Diabetes Mellitus Tipo 2 , Humanos , Masculino , Femenino , Persona de Mediana Edad , Estudios de Casos y Controles , Diabetes Mellitus Tipo 2/complicaciones , Resultado del Tratamiento , Implantes Dentales , Hemoglobina Glucada , Anciano , Implantación Dental Endoósea/métodos , Pérdida de Hueso Alveolar/diagnóstico por imagen , Índice Periodontal , Adulto , Hiperglucemia
9.
Catheter Cardiovasc Interv ; 102(4): 641-645, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37622612

RESUMEN

Coronavirus disease 2019 (COVID-19) increases the risk of ST-segment elevation myocardial infarction (STEMI), and is associated with a higher occurrence of nonobstructive coronary artery disease. We present a unique case of STEMI with concomitant COVID-19 infection in a young female found to have slow flow in multiple vessels on angiography, likely due to microvascular thrombi. Three months later, the patient developed coronary microvascular dysfunction (CMD), suggesting an evolution of microvascular thrombi and injury into subsequent CMD.

10.
Catheter Cardiovasc Interv ; 101(1): 1-10, 2023 01.
Artículo en Inglés | MEDLINE | ID: mdl-36423258

RESUMEN

OBJECTIVES: To assess the characteristics and prognosis of ST-elevation myocardial infarction (STEMI) patients, presenting between 12 and 24 h after symptom onset, in contemporary regional STEMI systems of care in the United States. BACKGROUND: Previous observational studies have been inconsistent regarding the benefit of primary percutaneous coronary intervention (PCI) compared with conservative management for late-presenting STEMI patients and the majority of randomized trials are from the fibrinolytic era. METHODS: Using a two-center registry-based cohort from March 2003 to December 2020, we evaluated the frequency, clinical characteristics, and outcomes of STEMI patients, stratified by symptom onset to balloon time: <3, 3-6, 6-12, and 12-24 h (late presenters). RESULTS: Among 5427 STEMI patients with available symptom onset time, 6.2% were late presenters, which increased to 11% during the early phase of the Covid-19 pandemic. As symptom onset to balloon time increased, patients were more likely to be older, female, and have a history of hypertension and diabetes mellitus. Late presenters with an identifiable culprit lesion were less likely to be revascularized with PCI (96%, 96%, 95%, and 92%; p for trend = 0.004) and had a longer median door-to-balloon time (82, 109, 107, and 117 min; p for trend < 0.001). In-hospital and 1-year death risks were comparable between late and earlier presenters. CONCLUSION: Despite the unfavorable risk profile and longer door-to-balloon time, clinical outcomes of late presenters were similar to those presenting within 12 h of symptom onset.


Asunto(s)
COVID-19 , Intervención Coronaria Percutánea , Infarto del Miocardio con Elevación del ST , Humanos , Femenino , Infarto del Miocardio con Elevación del ST/diagnóstico por imagen , Infarto del Miocardio con Elevación del ST/terapia , Intervención Coronaria Percutánea/efectos adversos , Pandemias , Resultado del Tratamiento , COVID-19/diagnóstico
11.
BMC Infect Dis ; 23(1): 564, 2023 Aug 29.
Artículo en Inglés | MEDLINE | ID: mdl-37644383

RESUMEN

BACKGROUND: Gram-negative bloodstream infections (GN-BSIs) are a significant clinical challenge. The utility of follow-up blood cultures (FUBCs) in GN-BSIs and their impact on mortality and antibiotic consumption are areas of debate. This study aimed to evaluate the effect of FUBCs on mortality and antibiotic consumption in patients with GN-BSIs. METHODS: This single-center, retrospective study was conducted in aged > 18 years of patients with GN-BSIs. FUBC was defined as a blood culture performed 2-7 days after the first blood culture. Patients were grouped as FUBC and no FUBC and compared. A 1:1 match analysis was performed between the groups according to the SOFA score. The matched subgroup was compared for mortality risk factors with logistic regression models. The two groups were compared for the duration of effective antibiotic therapy and total antibiotic consumption (days of therapy per 1000 patient days (DOT/1000 PD)). RESULTS: FUBC was performed in 564 (69.4%) of 812 patients. Persistent, positive and negative FUBC rates were 7.9%, 14%, and 78%, respectively. The frequency of persistent GN-BSI in patients with appropriate antibiotic therapy was 3.9%. SOFA score (OR:1.33; 95% CI, 1.23-1.44), Charlson comorbidity index score (OR:1.18; 95% CI, 1.08-1.28), hospital-acquired infections (OR:1.93; 95% CI, 1.08-3.46) and carbapenem-resistant GN-BSI (OR: 2.92; 95% CI, 1.72-4.96) were independent risk factors for mortality. No relationship was found between FUBC and mortality (p > 0.05). Duration of effective antibiotic therapy (10(4-16) vs. 15(9-20), p < 0.001) and DOT/1000 PD (1609 (1000-2178) vs. 2000 (1294-2769), p < 0.001) were longer in the FUBC group. CONCLUSION: Routine FUBC should not be recommended because of the low prevalence of persistent infections in patients under appropriate antibiotic therapy and FUBC increases antibiotic consumption.


Asunto(s)
Antibacterianos , Sepsis , Humanos , Antibacterianos/uso terapéutico , Cultivo de Sangre , Estudios de Seguimiento , Estudios Retrospectivos
12.
Pediatr Nephrol ; 38(11): 3699-3709, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37221349

RESUMEN

BACKGROUND: Children with lupus have a higher chance of nephritis and worse kidney outcome than adult patients. METHODS: We retrospectively analyzed clinical presentation, treatment and 24-month kidney outcome in a cohort of 382 patients (≤ 18 years old) with lupus nephritis (LN) class ≥ III diagnosed and treated in the last 10 years in 23 international centers. RESULTS: The mean age at onset was 11 years 9 months and 72.8% were females. Fifty-seven percent and 34% achieved complete and partial remission at 24-month follow-up, respectively. Patients with LN class III achieved complete remission more often than those with classes IV or V (mixed and pure). Only 89 of 351 patients maintained stable complete kidney remission from the 6th to 24th months of follow-up. eGFR ≥ 90 ml/min/1.73 m2 at diagnosis and biopsy class III were predictive of stable kidney remission. The youngest and the oldest age quartiles (2y-9y, 5m) (14y, 2m-18y,2m) showed lower rates of stable remission (17% and 20.7%, respectively) compared to the two other age groups (29.9% and 33.7%), while there was no difference in gender. No difference in achieving stable remission was found between children who received mycophenolate or cyclophosphamide as induction treatment. CONCLUSION: Our data show that the rate of complete remission in patients with LN is still not high enough. Severe kidney involvement at diagnosis was the most important risk factor for not achieving stable remission while different induction treatments did not impact outcome. Randomized treatment trials involving children and adolescents with LN are needed to improve outcome for these children. A higher resolution version of the Graphical abstract is available as Supplementary information.


Asunto(s)
Nefritis Lúpica , Adolescente , Niño , Femenino , Humanos , Masculino , Ciclofosfamida/uso terapéutico , Inmunosupresores/uso terapéutico , Riñón/patología , Nefritis Lúpica/diagnóstico , Nefritis Lúpica/tratamiento farmacológico , Nefritis Lúpica/patología , Ácido Micofenólico/uso terapéutico , Inducción de Remisión , Estudios Retrospectivos , Resultado del Tratamiento
13.
Eur J Pediatr ; 182(12): 5473-5482, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37777601

RESUMEN

Despite the advanced knowledge concerning autoinflammatory diseases (AID), more data regarding the optimal treatment options and outcomes of the children who met the criteria of more than one AID are required. This study aimed to describe the demographic and clinical characteristics of children from familial Mediterranean fever (FMF)-endemic countries who meet both the FMF and the periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome criteria. Moreover, we aimed to measure the response rates to colchicine and tonsillectomy and evaluate the factors affecting the colchicine response in these patients. The study was conducted at pediatric rheumatology tertiary centre. A total of 131 patients (58 females; 73 males) who met both the modified Marshall and pediatric FMF criteria were included. The median age at onset was 18 months (1-77 months), and the mean age at diagnosis was 47 ± 21.88 months. The median interval between episodes was 21 (7-90) days. The median disease duration was 46 (6-128) months. Consanguineous marriage was detected in 17 (13%) of the patients. The most common clinical finding was fever (100%), followed by exudative pharyngitis (88.5%), abdominal pain (86.3%), arthralgia (61.8%), stomatitis (51.1%), adenitis (42%), myalgia (28.7%), chest pain (16%), maculopapular rash (12.2%), arthritis (8.4%), and erysipelas-like rash (4.6%). MEFV gene variants were identified in 106 (80.9%) patients. The most common variants were M694V heterozygous (29%). We found that patients with tonsillopharyngitis, aphthous stomatitis, and PFAPA family history were more likely to be colchicine-resistant and tonsillectomy responsive, while those with exon 10 MEFV gene mutations were more prone to have a favorable response to colchicine.     Conclusion: PFAPA syndrome patients with exon 10 MEFV gene mutation, showing typical FMF symptoms, should be treated with colchicine, even after tonsillectomy. In multivariate analysis, PFAPA family history and lack of exon 10 MEFV gene mutations were independent risk factors for colchicine resistance. Thus, tonsillectomy may be recommended as a possible treatment option for these patients. It has yet to be clarified when colchicine treatment will be discontinued in patients whose attacks ceased after tonsillectomy that was performed due to colchicine unresponsiveness. What is Known: • A certain number of patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome concomitantly fulfill the familial Mediterranean fever (FMF) criteria. • While colchicine is proposed as a first treatment choice in familial Mediterranean fever (FMF), corticosteroids are recommended as a first-line treatment in PFAPA syndrome patients. What is New: • In patients with concomitant PFAPA syndrome and FMF, PFAPA family history and lack of exon 10 MEFV gene mutation are predictive factors of colchicine resistance. • The presence of exon 10 MEFV gene mutations in patients with concomitant FMF and PFAPA syndrome has a favourable effect on response to colchicine treatment.


Asunto(s)
Exantema , Fiebre Mediterránea Familiar , Linfadenitis , Linfadenopatía , Faringitis , Estomatitis Aftosa , Tonsilectomía , Masculino , Femenino , Niño , Humanos , Lactante , Preescolar , Fiebre Mediterránea Familiar/complicaciones , Fiebre Mediterránea Familiar/diagnóstico , Fiebre Mediterránea Familiar/tratamiento farmacológico , Estomatitis Aftosa/diagnóstico , Fiebre/diagnóstico , Faringitis/diagnóstico , Linfadenitis/diagnóstico , Colchicina/uso terapéutico , Síndrome , Exantema/complicaciones , Exantema/tratamiento farmacológico , Pirina/genética
14.
Rheumatol Int ; 43(1): 137-145, 2023 01.
Artículo en Inglés | MEDLINE | ID: mdl-36116090

RESUMEN

Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome is one of the most common autoinflammatory fever disorders in the childhood which may co-exists with familial Mediterranean fever (FMF) causing treatment complexity. As the role of surgery in PFAPA syndrome is still controversial, in this paper, our aim is to present our results of tonsillectomy/adenotonsillectomy in the treatment of PFAPA syndrome. Archives of a tertiary care hospital were investigated for patients who underwent tonsillectomy or adenotonsillectomy due to PFAPA Syndrome between 2010 and 2020. 344 patients were found but only 281 of them were accessible. Through phone call interview and chart review methods, preoperative and postoperative the number and severity of the attacks and general satisfaction after the operation were recorded and analyzed. Also, patients with concomitant FMF were analyzed separately. A total of 281 patients were included in the study. There was no improvement in 10 (3.55%) patients. Eight (2.84%) patients showed mild improvement, 29 (10.32%) patients had moderate improvement and 234 (83.27%) patients had full recovery after tonsillectomy. There were 266 PFAPA patients without FMF. No improvement, mild improvement, moderate improvement, and full recovery in this patient group were 5 (1.9%), 6 (2.3%), 25 (9.4%) and 230 (86.5%), respectively. FMF was present in 5.33% (15/281) of the patients. In PFAPA + FMF group 5 patients had no improvement (33.3%), 2 had mild improvement (13.3%), 4 had moderate improvement (26.7%) and 4 had full recovery (26.7%). Benefit of tonsillectomy was significantly lower in the patients with concomitant FMF when compared to the patients who did not have FMF (p < 0.001). Age of diagnosis, age of operation, severity of the disease, type of operation, and gender were found to have no significant relationship with the benefit from surgery (p < 0.05). According to the findings of this study, tonsillectomy is an effective long-term treatment for PFAPA syndrome with success rate of 83.27%. Also, preoperatively FMF should be considered in these patients, which dramatically reduces surgical efficacy.


Asunto(s)
Fiebre Mediterránea Familiar , Linfadenitis , Linfadenopatía , Faringitis , Estomatitis Aftosa , Tonsilectomía , Humanos , Niño , Tonsilectomía/métodos , Fiebre Mediterránea Familiar/complicaciones , Fiebre Mediterránea Familiar/diagnóstico , Fiebre Mediterránea Familiar/cirugía , Estomatitis Aftosa/complicaciones , Estomatitis Aftosa/cirugía , Estomatitis Aftosa/diagnóstico , Faringitis/complicaciones , Faringitis/cirugía , Faringitis/diagnóstico , Fiebre/cirugía , Fiebre/complicaciones , Linfadenopatía/complicaciones , Linfadenitis/complicaciones , Linfadenitis/diagnóstico , Linfadenitis/cirugía , Síndrome
15.
Rheumatol Int ; 2023 Jun 06.
Artículo en Inglés | MEDLINE | ID: mdl-37277620

RESUMEN

The aim of this retrospective study is to evaluate the efficacy of a single-dose anakinra during familial Mediterranean fever (FMF) attacks and its effect on the duration, severity, and frequency of attacks. The patients with FMF who had disease episode and received a single-dose anakinra during disease episode between December 2020 and May 2022 were included. Demographic characteristics, MEFV gene variants detected, concomitant medical conditions, demographics of recent and previous episodes, laboratory findings and length of hospital stay were recorded. A retrospective analysis of medical records revealed 79 attacks from 68 patients who met inclusion criteria. The patients had a median age of 13 (2.5-25) years. All patients reported that the average duration of their previous episodes lasted longer than 24 h. When the recovery time of attacks after subcutaneous anakinra application at the disease attack was examined, it was observed that 4 attacks (5.1%) ended in 10 min; 10 attacks (12.7%) in 10-30 min; 29 attacks (36.7%) in 30-60 min; 28 attacks (35.4%) in 1-4 h; 4 attacks (5.1%) in 24 h; and 4 attacks (5.1%) ended in more than 24 h. There was no patient who did not recover from their attack after a single dose of anakinra. Although the efficacy of a single-dose anakinra administration during FMF attacks in children needs to be confirmed by prospective studies, our results suggest that use of a single-dose anakinra during FMF attacks is effective in reduction of severity and duration of disease attacks.

16.
J Sports Sci ; 41(14): 1372-1382, 2023 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-37856689

RESUMEN

The primary aim was to compare the peak running speed (PRS) attained in the 40-m linear sprint test, in an analytical-based soccer drill, in the 5-0-5 test, and a training match scenario. The secondary aim of the study was to evaluate the differences between the three assessment sessions and identify how the tests can vary from session to session. Additionally, we aimed to investigate the within-test variability to understand how consistent the performance is within each test format across the different sessions. Forty male under-19 players competing at the national level participated in this study. A training session was observed for each of the three study weeks in which the following tests/scenarios were monitored using a GPS. The 40-m linear sprint test and the analytical-based soccer drill presented the smallest within-subject coefficients of variation between the sessions. A large correlation (r = 0.742) was found between the PRS during the 40-m linear sprint test and the analytical-based soccer drill. The 40-m linear sprint test was the best method of those examined for measuring PRS. The analytical drill provides a reliable method for measuring PRS, although it differs from the 40-metre linear sprint test.


Asunto(s)
Rendimiento Atlético , Carrera , Fútbol , Humanos , Masculino , Adulto Joven , Investigación
17.
Heart Fail Clin ; 19(2S): e1-e8, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-37169437

RESUMEN

The interplay of COVID-19 and heart failure is complex and involves direct and indirect effects. Patients with existing heart failure develop more severe COVID-19 symptoms and have worse clinical outcomes. Pandemic-related policies and protocols have negatively affected care for cardiovascular conditions and established hospital protocols, which is particularly important for patients with heart failure.


Asunto(s)
COVID-19 , Enfermedades Cardiovasculares , Insuficiencia Cardíaca , Humanos , COVID-19/complicaciones , COVID-19/epidemiología , SARS-CoV-2 , Insuficiencia Cardíaca/epidemiología , Insuficiencia Cardíaca/terapia
18.
Heart Fail Clin ; 19(2): 221-229, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36863814

RESUMEN

The global health crisis caused by the COVID-19 pandemic has evolved rapidly to overburden health care organizations around the world and has resulted in significant morbidity and mortality. Many countries have reported a substantial and rapid reduction in hospital admissions for acute coronary syndromes and percutaneous coronary intervention. The reasons for such abrupt changes in health care delivery are multifactorial and include lockdowns, reduction in outpatient services, reluctance to seek medical attention for fear of contracting the virus, and restrictive visitation policies adopted during the pandemic. This review discusses the impact of COVID-19 on important aspects of acute MI care.


Asunto(s)
COVID-19 , Atención a la Salud , Infarto del Miocardio , Humanos , Atención Ambulatoria/estadística & datos numéricos , Control de Enfermedades Transmisibles/estadística & datos numéricos , COVID-19/epidemiología , Infarto del Miocardio/epidemiología , Infarto del Miocardio/terapia , Pandemias , Atención a la Salud/estadística & datos numéricos , Accesibilidad a los Servicios de Salud/estadística & datos numéricos
19.
Turk J Med Sci ; 53(4): 979-989, 2023 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38031949

RESUMEN

BACKGROUND: The aim of our study was to evaluate the long-term impacts of Kawasaki disease on our patients regarding coronary involvement demographic characteristics, treatment regimens, and clinical course. METHODS: Our study included 104 patients diagnosed and hospitalized with Kawasaki disease in our center, from January 2004 to January 2019. In our study, patients were divided into three groups according to coronary artery involvement. Patients in group 1 had no echocardiographic findings, while the ones in group 2 had coronary artery dilatation and ones in group 3 had coronary artery aneurysm (CAA). RESULTS: Among 104 patients, the median age was 9.15 (3.0-22.0) years, and 61 of the patients were male while 43 of the patients were female. With a wide range of 1.50-16.50 years of follow-up time, the median diagnosis age of our patients was 31 months (3.0-164.0). Fever duration (median day 10 (5-21), p = 0.025) was statistically significantly higher in group 3. Blood C-reactive protein (CRP) levels, white blood cell (WBC) counts, and neutrophil counts were significantly higher in group 3. There was a statistically significant difference between patients in group 3 and group 2 in which the lowest strain deformation values were in the patients of group 3. In contrast to group 1, the time for initiation of IVIG therapy is significantly prolonged both in group 2 (median: 9.5 days, p = 0.028) and group 3 (median: 10 days, p = 0.036). DISCUSSION: In our study, serum CRP levels, WBC count, and neutrophil count were higher in patients with coronary artery abnormalities, in agreement with the previous studies. In the light of our results, we consider that the most important determining factor for the development of coronary artery aneurysm is the time of intravenous immunoglobulin (IVIG) administration.


Asunto(s)
Aneurisma Coronario , Enfermedad de la Arteria Coronaria , Síndrome Mucocutáneo Linfonodular , Humanos , Niño , Masculino , Femenino , Lactante , Preescolar , Inmunoglobulinas Intravenosas/uso terapéutico , Síndrome Mucocutáneo Linfonodular/complicaciones , Síndrome Mucocutáneo Linfonodular/diagnóstico , Síndrome Mucocutáneo Linfonodular/epidemiología , Estudios Retrospectivos , Aneurisma Coronario/diagnóstico por imagen , Aneurisma Coronario/epidemiología , Aneurisma Coronario/etiología , Enfermedad de la Arteria Coronaria/epidemiología
20.
Biol Sport ; 40(4): 959-965, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37867758

RESUMEN

This study aimed to analyze within- and between-player variations of peak speed and sprinting actions occurring in small-sided games (SSGs: 1 v 1 and 5 v 5). The study followed a cohort study design. Twenty male youth soccer players (age: 17 years old) from the same team were observed over four consecutive weeks. Each week, the players participated in two sessions (day one and day two) during which SSGs were applied. The 1 vs. 1 format was employed with four repetitions of thirty seconds interspaced with two-minute rest intervals, while the 5 vs. 5 format with four repetitions of four minutes and two-minute rest intervals between them. The players were monitored during all training sessions with the Polar Team Pro. The peak speed attained in each game, and the number of sprints were extracted as the primary outcomes. The between-player variability revealed a lower coefficient of variations for peak speed in the 1 vs. 1 (13.9%) and 5 vs. 5 (10.9%) formats than for sprints (1 v 1: 64.7%; 5 v 5: 65.5%). Considering the within-player variability, it was observed that sprints were more variable (1 vs. 1: 62.1%; 5 v 5: 65.7%) than peak speed (1 vs. 1: 16.4%; 5 v 5: 14.0%). The between-session analysis revealed that during week 1 (day 1), peak speed was significantly higher than during week 3 (day 1) in the 1 vs. 1 format (+3.0 km/h; p = 0.031; d = 1.296). Moreover, peak speed during week 3 (day 2) was considerably lower than during week 1 (-5.9 km/h; p < 0.001; d = 1.686) and week 2 (-5.0 km/h; p = 0.001; d = 1.639). The between-session analysis showed no significant differences in the sprint between the sessions on day 1 (p > 0.05). However, on day two, the sprint was substantially higher during week one than during week four in the 5 vs. 5 format (+5.40 n; p = 0.002; d = 2.571). In conclusion, this study revealed that peak speed presents lower within- and between-player variability than the number of sprints. Considering these two measures, there are no considerable variations between the weeks. Coaches should consider identifying strategies to stabilize the stimulus regarding the number of sprints if this represents one of the targets for employing SSGs.

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