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1.
Am J Otolaryngol ; 42(3): 102903, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33485050

RESUMEN

OBJECTIVE: PFAPA syndrome is derived from the initials of the English words of the findings that make up the syndrome ("Periodic Fever", "Aphthous Stomatitis", "Pharyngitis", "Adenitis"). This study aims to evaluate the vestibular system in patients with PFAPA syndrome by the cVEMP test and to give a general review of PFAPA syndrome in light of current literature. METHODS: In this prospective study, 30 patients aged 4-6 who were diagnosed with PFAPA in a tertiary pediatrics clinic, between January 2016 and February 2020 and 30 children of the same age group who applied to a tertiary otorhinolaryngology clinic for other reasons and proven to have no hearing or vestibular problems were included and in addition to routine physical examination, electromyographic activity of the sternocleidomastoid muscle surface was measured. RESULTS: We found that the amplitude difference between cVEMP p1-n1 in patients with PFAPA syndrome in both ears decreased compared to the healthy control group. CONCLUSION: Our study proves there is a vestibular system involvement of PFAPA syndrome. This study is the first in the literature to search the relationship between PFAPA and the vestibular system.


Asunto(s)
Fiebre/etiología , Linfadenitis/etiología , Periodicidad , Faringitis/etiología , Estomatitis Aftosa/etiología , Enfermedades Vestibulares/complicaciones , Enfermedades Vestibulares/diagnóstico , Pruebas de Función Vestibular/métodos , Vestíbulo del Laberinto/fisiopatología , Factores de Edad , Niño , Preescolar , Electromiografía , Femenino , Fiebre/fisiopatología , Humanos , Linfadenitis/fisiopatología , Masculino , Faringitis/fisiopatología , Estudios Prospectivos , Estomatitis Aftosa/fisiopatología , Síndrome , Enfermedades Vestibulares/fisiopatología
2.
Eur Radiol ; 30(8): 4475-4485, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-32189052

RESUMEN

OBJECTIVES: To establish a diagnostic tree analysis (DTA) model based on ultrasonography (US) findings and clinical characteristics for differential diagnosis of common causes of cervical lymphadenopathy in children. METHODS: A total of 242 patients (131 boys, 111 girls; mean age, 11.2 ± 0.3 years; range, 1 month-18 years) with pathologically confirmed Kikuchi disease (n = 127), reactive hyperplasia (n = 64), lymphoma (n = 24), or suppurative lymphadenitis (n = 27) who underwent neck US were included. US images were retrospectively reviewed to assess lymph node (LN) characteristics, and clinical information was collected from patient records. DTA models were created using a classification and regression tree algorithm on the basis of US imaging and clinical findings. The patients were randomly divided into training (70%, 170/242) and validation (30%, 72/242) datasets to assess the diagnostic performance of the DTA models. RESULTS: In the DTA model based on all predictors, perinodal fat hyperechogenicity, LN echogenicity, and short diameter of the largest LN were significant predictors for differential diagnosis of cervical lymphadenopathy (overall accuracy, 85.3% and 83.3% in the training and validation datasets). In the model based on categorical parameters alone, perinodal fat hyperechogenicity, LN echogenicity, and loss of fatty hilum were significant predictors (overall accuracy, 84.7% and 86.1% in the training and validation datasets). CONCLUSIONS: Perinodal fat hyperechogenicity, heterogeneous echotexture, short diameter of the largest LN, and loss of fatty hilum were significant US findings in the DTA for differential diagnosis of cervical lymphadenopathy in children. KEY POINTS: • Diagnostic tree analysis model based on ultrasonography and clinical findings would be helpful in differential diagnosis of pediatric cervical lymphadenopathy. • Significant predictors were perinodal fat hyperechogenicity, heterogeneous echotexture, short diameter of the largest LN, and loss of fatty hilum.


Asunto(s)
Algoritmos , Diagnóstico Diferencial , Linfadenitis Necrotizante Histiocítica/diagnóstico por imagen , Linfadenopatía/diagnóstico por imagen , Linfoma/diagnóstico por imagen , Seudolinfoma/diagnóstico por imagen , Adolescente , Biopsia con Aguja Fina , Biopsia con Aguja Gruesa , Niño , Preescolar , Eritema/fisiopatología , Femenino , Fiebre/fisiopatología , Linfadenitis Necrotizante Histiocítica/patología , Linfadenitis Necrotizante Histiocítica/fisiopatología , Humanos , Procesamiento de Imagen Asistido por Computador , Lactante , Ganglios Linfáticos/diagnóstico por imagen , Ganglios Linfáticos/patología , Linfadenitis/diagnóstico por imagen , Linfadenitis/patología , Linfadenitis/fisiopatología , Linfadenopatía/patología , Linfadenopatía/fisiopatología , Linfoma/patología , Linfoma/fisiopatología , Masculino , Cuello , Seudolinfoma/patología , Seudolinfoma/fisiopatología , Reproducibilidad de los Resultados , Estudios Retrospectivos , Ultrasonografía , Ultrasonografía Doppler en Color
3.
Rheumatol Int ; 40(1): 91-96, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31346686

RESUMEN

The periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an auto-inflammatory condition characterized by recurrent episodes of fever accompanied by aphthosis, cervical adenitis, and pharyngitis. Diagnosis of PFAPA could be challenging due to clinic overlap with familial Mediterranean fever (FMF). An international consensus has been established recently, to define a new set of classification criteria for PFAPA syndrome. We aimed to evaluate the performance of recently proposed PFAPA criteria, to assess their utility in FMF regions. Patients diagnosed with PFAPA syndrome, FMF, and juvenile idiopathic arthritis (JIA) were included. Two investigators blindly evaluated all of patients for the newly proposed PFAPA criteria. A total of 542 patients (322 with PFAPA syndrome, 118 FMF and 102 JIA) were evaluated. Mean age of patients was 6.6 ± 2.81, 12.75 ± 3.9, and 12.42 ± 4.8 years for PFAPA, FMF, and JIA, respectively. We found quite high sensitivity (89.7%) but insufficient specificity of newly proposed PFAPA criteria (69.5%). When applied to control patients separately, specificity was found to be 61% and 79.4% for FMF and JIA patients, respectively. Positive predictive value was 81%, while negative predictive value was 82%. Recently proposed PFAPA criteria have satisfactory sensitivity, but its specificity is still under expectation. There is a need for a distinctive criterion between PFAPA syndrome and FMF, in FMF endemic regions, e.g., cryptic tonsillitis rapidly responsive to single dose of glucocorticoids. Further studies with higher patients' number in different regions are needed.


Asunto(s)
Artritis Juvenil/diagnóstico , Fiebre Mediterránea Familiar/diagnóstico , Fiebre/fisiopatología , Enfermedades Autoinflamatorias Hereditarias/diagnóstico , Linfadenitis/fisiopatología , Faringitis/fisiopatología , Estomatitis Aftosa/fisiopatología , Adolescente , Artritis Juvenil/clasificación , Artritis Juvenil/fisiopatología , Estudios de Casos y Controles , Niño , Preescolar , Diagnóstico Diferencial , Enfermedades Endémicas , Fiebre Mediterránea Familiar/clasificación , Fiebre Mediterránea Familiar/fisiopatología , Femenino , Fiebre/complicaciones , Enfermedades Autoinflamatorias Hereditarias/clasificación , Enfermedades Autoinflamatorias Hereditarias/fisiopatología , Humanos , Linfadenitis/complicaciones , Masculino , Cuello , Faringitis/complicaciones , Estomatitis Aftosa/complicaciones , Síndrome
4.
Microb Pathog ; 135: 103628, 2019 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31325572

RESUMEN

Caseous lymphadenitis is an infectious disease of almost all animals, particularly small ruminants that are caused by Corynebacterium pseudotuberculosis. The organism causes the formation of suppurative abscesses in superficial and visceral lymph nodes and in visceral organs. This current study was designed to elucidate the clinicopathological responses and PCR detection of the aetiological agent in the vital organs of goats challenged with C. pseudotuberculosis and its immunogenic mycolic acid extract. A total of twelve clinically healthy crossbred Boer female goats were divided into three groups: A, B, and C (four goats per group). Group A was inoculated intradermally with 2 ml of sterile phosphate buffered saline (PBS) pH 7 as a control group. Group B was inoculated intradermally with 2 ml of mycolic acid extract (1 g/ml), while group C was inoculated intradermally with 2 ml of 109 colony-forming unit (cfu) of live C. pseudotuberculosis. The experimental animals were observed for clinical responses for 90 days post-inoculation and the clinical signs were scored according to the severity. The clinical signs observed in this study were temperature, heart rate, respiratory rate, rumen motility, enlargement of lymph nodes, and body condition score. The experimental animals were euthanised and tissue samples from different anatomical regions of the vital organs were collected in 10% buffered formalin, processed, sectioned, and stained with H&E. Results of both C. pseudotuberculosis and mycolic acid treated groups indicated a significant difference (p < 0.05) in body temperature. Group C showed a significant increase in temperature (p < 0.05) at week 1 (39.59 ±â€¯0.29 °C), week 2 (39.67 ±â€¯0.27 °C) and week 3 (40.22 ±â€¯0.15 °C). Whereas group B showed a significant increase in temperature (p < 0.05) only at week 1 (39.36 ±â€¯0.14 °C). Heart rate in group C showed a significant increase between week 1 (93.35 ±â€¯0.42 bpm) and week 11 (86.52 ±â€¯1.32 bpm), and the mean heart rate of group B showed a significant increase (p < 0.05) between week 1 (89.90 ±â€¯0.60 bpm) and week 9 (86.90 ±â€¯0.99 bpm). Group C showed a significant increase of respiratory rate (p < 0.05) at week 1 (36.85 ±â€¯0.14 bpm), week 2 (36.90 ±â€¯0.62), week 3 (30.80 ±â€¯1.97 bpm), and week 4 (34.85 ±â€¯1.19 bpm). The mean of the respiratory rate of group B only increased at week 1 (32.98 ±â€¯1.30 bpm) and week 2 (31.87 ±â€¯0.48 bpm). Both groups C & B showed significant decreases in rumen motility and body condition score as compared to the control. The histopathological changes were significant in group C, this was shown by mild to severe haemorrhage, congestion, degeneration and necrosis, oedema, infiltration with inflammatory cells mainly lymphocytes and macrophages, while group B was less affected and showed mild to moderate haemorrhage, congestion, degeneration and necrosis, infiltration of inflammatory cells and oedema as compared to the control group. This study concluded that C. pseudotuberculosis caused typical CLA disease with a short incubation period in the experiment. While the mycolic acid extracted from C. pseudotuberculosis caused mild clinical signs, no abscess formation, and negative PCR result. Moreover, evidence of mild to moderate histopathological changes in vital organs was also observed.


Asunto(s)
Infecciones por Corynebacterium/diagnóstico , Infecciones por Corynebacterium/microbiología , Corynebacterium pseudotuberculosis/aislamiento & purificación , Corynebacterium pseudotuberculosis/metabolismo , Enfermedades de las Cabras/diagnóstico , Enfermedades de las Cabras/microbiología , Ácidos Micólicos/inmunología , Ácidos Micólicos/metabolismo , Absceso/microbiología , Animales , Temperatura Corporal , Infecciones por Corynebacterium/patología , Infecciones por Corynebacterium/fisiopatología , Corynebacterium pseudotuberculosis/genética , Femenino , Enfermedades de las Cabras/patología , Enfermedades de las Cabras/fisiopatología , Cabras , Corazón , Frecuencia Cardíaca , Riñón/patología , Recuento de Leucocitos , Hígado/patología , Pulmón/patología , Ganglios Linfáticos/microbiología , Linfadenitis/diagnóstico , Linfadenitis/inmunología , Linfadenitis/microbiología , Linfadenitis/fisiopatología , Reacción en Cadena de la Polimerasa/métodos , Frecuencia Respiratoria , Bazo/patología
5.
J Immunol ; 197(6): 2338-52, 2016 09 15.
Artículo en Inglés | MEDLINE | ID: mdl-27511736

RESUMEN

Type I IFN (IFN-α/ß)-driven immune responses to acute viral infection are critical to counter replication and prevent dissemination. However, the mechanisms underlying host resistance to HSV type 1 (HSV-1) are incompletely understood. In this study, we show that mice with deficiencies in IFN-α/ß signaling or stimulator of IFN genes (STING) exhibit exacerbated neurovirulence and atypical lymphotropic dissemination of HSV-1 following ocular infection. Synergy between IFN-α/ß signaling and efficacy of early adaptive immune responses to HSV-1 were dissected using bone marrow chimeras and adoptive cell transfer approaches to profile clonal expansion, effector function, and recruitment of HSV-specific CD8(+) T cells. Lymphotropic viral dissemination was commensurate with abrogated CD8(+) T cell responses and pathological alterations of fibroblastic reticular cell networks in the draining lymph nodes. Our results show that resistance to HSV-1 in the trigeminal ganglia during acute infection is conferred in part by STING and IFN-α/ß signaling in both bone marrow-derived and -resident cells, which coalesce to support a robust HSV-1-specific CD8(+) T cell response.


Asunto(s)
Linfocitos T CD8-positivos/inmunología , Herpes Simple/virología , Herpesvirus Humano 1/inmunología , Interferón beta-1a/metabolismo , Interferón-alfa/metabolismo , Linfadenitis/inmunología , Linfadenitis/virología , Proteínas de la Membrana/metabolismo , Inmunidad Adaptativa , Traslado Adoptivo , Animales , Linfocitos T CD8-positivos/patología , Ojo/virología , Herpes Simple/inmunología , Herpesvirus Humano 1/patogenicidad , Herpesvirus Humano 1/fisiología , Interferón beta-1a/genética , Interferón beta-1a/inmunología , Interferón-alfa/genética , Interferón-alfa/inmunología , Linfadenitis/fisiopatología , Proteínas de la Membrana/deficiencia , Ratones , Transducción de Señal , Ganglio del Trigémino/inmunología , Ganglio del Trigémino/fisiopatología , Ganglio del Trigémino/virología , Replicación Viral
6.
Vet Pathol ; 50(2): 318-23, 2013 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-22732360

RESUMEN

Caseous lymphadenitis is an infectious and contagious disease caused by Corynebacterium pseudotuberculosis, with a worldwide distribution and high prevalence in small ruminant populations. This disease causes significant economic loss in small ruminants through reduced meat, wool, and milk production. C. pseudotuberculosis can also affect horses, domestic and wild large ruminants, swine, and man. It is considered an occupational zoonosis for humans. As part of in vitro investigations of the pathogenesis of C. pseudotuberculosis, this study analyzed its capacity to adhere to and invade the FLK-BLV-044 cell line, derived from ovine embryonic kidney cells. C. pseudotuberculosis showed a measurable capacity to adhere to and invade this cell line with no significant differences between the four strains assessed. The incubation of the cell line at 4ºC, pre-incubation with sugars, complete and heat inactivated antiserum, and heat-killed and ultraviolet-killed bacteria produced a significant (P < 0.05) decrease in the invasion efficiency or inability to invade the cell line. Plate counting and fluorescence studies showed intracellular bacteria for up to 6 days. Non-phagocytic cells may therefore act as a suitable environment for C. pseudotuberculosis survival and play a role in the spread of infection and/or maintenance of a carrier state.


Asunto(s)
Adhesión Celular/fisiología , Corynebacterium pseudotuberculosis/fisiología , Embrión de Mamíferos/citología , Riñón/citología , Linfadenitis/veterinaria , Enfermedades de las Ovejas/microbiología , Enfermedades de las Ovejas/fisiopatología , Análisis de Varianza , Animales , Recuento de Células/veterinaria , Línea Celular , Corynebacterium pseudotuberculosis/genética , Corynebacterium pseudotuberculosis/patogenicidad , Gentamicinas , Linfadenitis/microbiología , Linfadenitis/fisiopatología , Microscopía Fluorescente , Ovinos , España , Especificidad de la Especie
7.
Arthritis Rheum ; 63(4): 1141-50, 2011 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-21225694

RESUMEN

OBJECTIVE: To analyze the long-term impact of the R92Q mutation of TNFRSF1A in children with periodic fever, in comparison with children with tumor necrosis factor receptor-associated periodic syndrome (TRAPS) with TNFRSF1A structural mutations and children with periodic fever of unknown origin fulfilling the criteria for periodic fever, aphthosis, pharyngitis, and adenitis syndrome (PFAPA). METHODS: The extracellular region of TNFRSF1A was analyzed in 720 consecutive children with periodic fever, using denaturing high-performance liquid chromatography and DNA sequencing. Followup data on 11 pediatric patients with TNFRSF1A structural mutations (cysteine or T50M), 23 pediatric patients with an R92Q substitution, and 64 pediatric patients with PFAPA were collected during routine clinic visits. The 50-item Child Health Questionnaire was used to assess health-related quality of life (HRQOL). RESULTS: The frequency of typical TRAPS-related clinical manifestations was significantly lower and the impact of the disease on HRQOL was significantly reduced in patients with the R92Q mutation compared with TRAPS patients carrying structural mutations of TNFRSF1A. Followup data on 11 TRAPS patients with TNFRSF1A structural mutations (mean followup 7.9 years), 16 patients with theR92Q substitution (mean followup 7.3 years), and 64 patients with PFAPA (mean followup 5.2 years) were available. Patients with R92Q mutations and patients with PFAPA displayed a higher rate of self-resolution or amelioration of the fever episodes than did TRAPS patients with structural mutations. CONCLUSION: Although some cases may progress to a more chronic disease course, the majority of children with an R92Q mutation of the TNFRSFA1 gene show a milder disease course than that in children with TNFRSFA1 structural mutations and have a high rate of spontaneous resolution and amelioration of the recurrent fever episodes.


Asunto(s)
Fiebre Mediterránea Familiar/genética , Fiebre/genética , Linfadenitis/genética , Mutación/genética , Faringitis/genética , Receptores Tipo I de Factores de Necrosis Tumoral/genética , Receptores del Factor de Necrosis Tumoral/fisiología , Adolescente , Antirreumáticos/uso terapéutico , Terapia Biológica , Niño , Preescolar , Fiebre Mediterránea Familiar/tratamiento farmacológico , Fiebre Mediterránea Familiar/fisiopatología , Femenino , Fiebre/tratamiento farmacológico , Fiebre/fisiopatología , Estudios de Seguimiento , Genotipo , Encuestas Epidemiológicas , Humanos , Lactante , Proteína Antagonista del Receptor de Interleucina 1/uso terapéutico , Estudios Longitudinales , Linfadenitis/tratamiento farmacológico , Linfadenitis/fisiopatología , Masculino , Faringitis/tratamiento farmacológico , Faringitis/fisiopatología , Calidad de Vida , Recurrencia , Estudios Retrospectivos , Esteroides/uso terapéutico , Síndrome
8.
Pediatr Infect Dis J ; 40(11): 1037-1045, 2021 11 01.
Artículo en Inglés | MEDLINE | ID: mdl-34636800

RESUMEN

BACKGROUND: There is no consensus on managing common adverse reactions to Bacille Calmette-Guérin (BCG) vaccination. We systematically reviewed the management of BCG-associated regional lymphadenitis and injection site abscess in immunocompetent individuals. METHODS: Searches of Medline, Embase and PubMed were done until November 2020. Randomized controlled trials (RCTs) and cohort studies that compared management strategies for complications of intradermal BCG vaccination were included. RESULTS: Of 1338 individual articles, 15 met inclusion criteria. Six RCTs, 4 prospective and 4 retrospective cohort studies compared management in 1022 children with BCG-associated lymphadenitis. For nonsuppurative lymphadenitis, no antimicrobial was found to significantly impact on time to resolution or prevention of suppuration. For suppurative lymphadenitis, there was some evidence that needle aspiration shortens time to resolution and prevents sinus tract formation. Surgical excision (mainly offered for persistent suppurative lymphadenitis) generally had favorable outcome. Two cohort studies (including 1 aforementioned) compared management strategies in up to 36 children with BCG injection site abscess; one showed no difference in outcome in children treated with antibiotics and the other reported complete resolution without treatment. CONCLUSIONS: Evidence does not support a role for antimicrobial therapy in the management of localized reactions to BCG vaccination in immunocompetent children. Needle aspiration may shorten the recovery period for BCG-associated suppurative lymphadenitis. BCG injection site abscess usually heals without treatment. However, studies are limited and cases are not well defined. Growing research into novel BCG applications provides opportunities to investigate optimal management strategies for adverse reactions in a prospective manner using active safety surveillance.


Asunto(s)
Absceso/etiología , Vacuna BCG/efectos adversos , Manejo de la Enfermedad , Inmunocompetencia , Linfadenitis/etiología , Humanos , Linfadenitis/fisiopatología , Ensayos Clínicos Controlados Aleatorios como Asunto
9.
Arch Pediatr ; 28(7): 580-582, 2021 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34511278

RESUMEN

Adenopathy in pediatrics can have many different causes: infectious, tumoral, and inflammatory. We report the case of an 8-year-old patient with a febrile popliteal ulceration associated with an inflammatory satellite inguinal lymph node adenitis. Serological tests and polymerase chain reaction analyses confirmed the diagnosis of ulceroglandular tularemia. An appropriate antimicrobial therapy led to a full recovery. This case reminds us to consider tularemia as a potential emergent disease in children presenting with subacute to chronic lymphadenopathy and thereby to choose the correct diagnostic tool and appropriate antimicrobial therapy.


Asunto(s)
Linfadenitis/etiología , Tularemia/complicaciones , Antibacterianos/uso terapéutico , Niño , Femenino , Humanos , Ganglios Linfáticos/anomalías , Ganglios Linfáticos/fisiopatología , Linfadenitis/fisiopatología , Tularemia/fisiopatología
10.
BMJ Case Rep ; 14(1)2021 Jan 11.
Artículo en Inglés | MEDLINE | ID: mdl-33431479

RESUMEN

Here we present the case of a 37-year-old previously healthy man who developed fever, headache and a unilateral, painful neck swelling while working offshore. He had no known contact with anyone with COVID-19; however, due to the ongoing pandemic, a nasopharyngeal swab was performed, which was positive for the virus. After transfer to hospital for assessment his condition rapidly deteriorated, requiring admission to intensive care for COVID-19 myocarditis. One week after discharge he re-presented with unilateral facial nerve palsy. Our case highlights an atypical presentation of COVID-19 and the multifaceted clinical course of this still poorly understood disease.


Asunto(s)
Alcalosis Respiratoria/sangre , Parálisis de Bell/fisiopatología , COVID-19/fisiopatología , Miocarditis/fisiopatología , Adulto , Alcalosis Respiratoria/etiología , Análisis de los Gases de la Sangre , Proteína C-Reactiva/metabolismo , COVID-19/sangre , COVID-19/terapia , Ecocardiografía , Edema/etiología , Electrocardiografía , Humanos , Hipotensión/etiología , Hipotensión/fisiopatología , Linfadenitis/etiología , Linfadenitis/fisiopatología , Imagen por Resonancia Magnética , Masculino , Miocarditis/sangre , Miocarditis/diagnóstico por imagen , Miocarditis/terapia , Péptido Natriurético Encefálico/sangre , Cuello , Terapia por Inhalación de Oxígeno , Fragmentos de Péptidos/sangre , Polipéptido alfa Relacionado con Calcitonina/sangre , Recuperación de la Función , SARS-CoV-2 , Troponina T/sangre , Vasoconstrictores/uso terapéutico
11.
Pediatr Rheumatol Online J ; 18(1): 31, 2020 Apr 15.
Artículo en Inglés | MEDLINE | ID: mdl-32293478

RESUMEN

BACKGROUND: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. There is considerable heterogeneity in management strategies and a lack of evidence-based treatment guidelines. Consensus treatment plans (CTPs) are standardized treatment regimens that are derived based upon best available evidence and current treatment practices that are a way to enable comparative effectiveness studies to identify optimal therapy and are less costly to execute than randomized, double blind placebo controlled trials. The purpose of this project was to develop CTPs and response criteria for PFAPA. METHODS: The CARRA PFAPA Working Group is composed of pediatric rheumatologists, infectious disease specialists, allergists/immunologists and otolaryngologists. An extensive literature review was conducted followed by a survey to assess physician practice patterns. This was followed by virtual and in-person meetings between 2014 and 2018. Nominal group technique (NGT) was employed to develop CTPs, as well as inclusion criteria for entry into future treatment studies, and response criteria. Consensus required 80% agreement. RESULTS: The PFAPA working group developed CTPs resulting in 4 different treatment arms: 1. Antipyretic, 2. Abortive (corticosteroids), 3. Prophylaxis (colchicine or cimetidine) and 4. Surgical (tonsillectomy). Consensus was obtained among CARRA members for those defining patient characteristics who qualify for participation in the CTP PFAPA study. CONCLUSION: The goal is for the CTPs developed by our group to lead to future comparative effectiveness studies that will generate evidence-driven therapeutic guidelines for this periodic inflammatory disease.


Asunto(s)
Fiebre/terapia , Linfadenitis/terapia , Faringitis/terapia , Estomatitis Aftosa/terapia , Corticoesteroides/uso terapéutico , Comités Consultivos , Antipiréticos/uso terapéutico , Niño , Preescolar , Cimetidina/uso terapéutico , Colchicina/uso terapéutico , Fiebre/fisiopatología , Antagonistas de los Receptores H2 de la Histamina/uso terapéutico , Humanos , Linfadenitis/fisiopatología , Cuello , Faringitis/fisiopatología , Estomatitis Aftosa/fisiopatología , Síndrome , Tonsilectomía , Moduladores de Tubulina/uso terapéutico
12.
PLoS One ; 13(9): e0203593, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30188947

RESUMEN

BACKGROUND: Early and proper treatment of tuberculosis could have an important impact on the morbidity, mortality and the economic situation of patients. There is insufficient knowledge on the extent of diagnostic delay and the associated factors in extrapulmonary tuberculosis (EPTB). The aims of this study were to assess the health care seeking behaviour, EPTB knowledge and diagnostic delay in presumptive EPTB patients at the main referral hospital in Zanzibar, factors associated with longer delay, and the impact of untreated EPTB on self-rated health. MATERIALS AND METHODS: Prospective data collection using a semi-structured questionnaire in patients presenting with symptoms suggestive of EPTB. The time between the onset of symptoms and first visit to a health care provider (patient delay), and then to the initiation of treatment (health system delay) and total delay were analysed according to sociodemographic and clinical factors and health care seeking trajectories. The EQ-5D-3L was used among the adult EPTB patients to assess the impact of treatment on self-rated health. RESULTS: Of the 132 patients with median age of 27 years (interquartile range 8-41), 69 were categorized as TB cases and 63 as non-TB cases. The median patient, health system and total delays were 14, 34 and 62 days respectively, among the EPTB patients. A longer health system delay with repeated visits to the same health care level was reported. Significantly better self-rated health status was described after treatment. The knowledge regarding extrapulmonary disease was low. CONCLUSION: Many EPTB patients, presenting to the main referral hospital in Zanzibar, experience a long delay in the initiation of treatment, specially patients with TB lymphadenitis. The health system delay is the major contributor to the total delay. The improvement of self-rated health after treatment implies that timely treatment has the potential to reduce morbidity and the economic loss for the patient.


Asunto(s)
Tuberculosis Pulmonar/diagnóstico , Adolescente , Adulto , Distribución de Chi-Cuadrado , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Linfadenitis/diagnóstico , Linfadenitis/fisiopatología , Masculino , Persona de Mediana Edad , Aceptación de la Atención de Salud , Estudios Prospectivos , Tanzanía , Tuberculosis Pulmonar/fisiopatología , Adulto Joven
13.
Orphanet J Rare Dis ; 13(1): 132, 2018 08 09.
Artículo en Inglés | MEDLINE | ID: mdl-30092788

RESUMEN

BACKGROUND: Conventionally, PFAPA syndrome is considered as a benign disease compared to other recurrent fevers because it completely passes before adulthood. However, in our clinical practice, fever episodes have a huge impact on daily activities. METHODS: Observational cohort study using the Pediatric Quality of Life Inventory (PedsQL™ 4.0) Generic Core and Fatigue Scales. PedsQL™ uses a modular approach to measure the HRQOL in children with acute and chronic health conditions. We used pediatric FMF patients as the control group. RESULTS: We included 33 children with PFAPA and compared them to 27 FMF patients matched for age: preschool-age children (2 to 7 years) and school-age children and youths (8 to18 years). PedsQL™ self-reported scores of children with PFAPA were systematically lower than those of FMF peers for general quality of life and physical and psychosocial functioning (significant only in the preschool-age group). PedsQL™ self-reported fatigue scores of children with PFAPA were significantly lower than those of FMF peers for both preschoolers and school-age children and youths. Parent proxy-reports were not significantly different, even though scores were systematically lower for the parents of PFAPA children. CONCLUSION: Our study demonstrates, for the first time, that the wellbeing of PFAPA children is poor, with a major impact on psychosocial functioning and increased fatigue. The quality of life of PFAPA children appears to be even lower than that of FMF patients, for whom a lower than normal HRQOL has already been demonstrated.


Asunto(s)
Linfadenitis/fisiopatología , Niño , Preescolar , Fiebre Mediterránea Familiar/complicaciones , Fiebre Mediterránea Familiar/fisiopatología , Femenino , Humanos , Linfadenitis/complicaciones , Masculino , Faringitis/complicaciones , Faringitis/fisiopatología , Psicometría , Calidad de Vida , Índice de Severidad de la Enfermedad
14.
Am J Surg Pathol ; 23(9): 1040-7, 1999 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-10478663

RESUMEN

Kikuchi's disease (KD) is an idiopathic, self-limited necrotizing lymphadenitis that can clinically and histologically mimic high-grade lymphoma, including Hodgkin's disease, or can be mistaken for the lymphadenitis of systemic lupus erythematosus (SLE). Involvement of extranodal sites is unusual but well documented, especially in Asia, where KD is more common than in North America or Europe. The successful distinction of KD from malignant lymphoma and SLE is imperative for the appropriate treatment of affected patients. We describe five patients with cutaneous involvement by KD, all of whom presented with fever, lymphadenopathy, and an eruption on the skin of the upper body, which in one case was clinically suspected to be due to SLE and in another, polymorphous light eruption. The patients ranged in age from 10 months to 42 years (median, 33 years) and included three females and two males. All five patients had negative serologic studies for collagen vascular disease. Each patient had a lymph node biopsy showing the typical necrotizing lymphadenitis of KD. Skin biopsies from all five patients shared a specific constellation of histologic features: vacuolar interface change with necrotic keratinocytes, a dense lymphohistiocytic superficial and deep perivascular and interstitial infiltrate, varying amounts of papillary dermal edema, and abundant karyorrhectic debris with a conspicuous absence of neutrophils and a paucity of plasma cells, paralleling the nodal histology in KD. CD68 immunohistochemistry on paraffin-embedded sections showed many histiocytes and plasmacytoid monocytes in all cases, whereas CD3, CD4, and CD8 showed highly variable staining among the cases. There was only rare staining with TIA-1 and CD30. We believe that the papular eruption of KD has recognizable histopathologic features and that a CD68 stain that marks many cells that initially seem to be lymphocytes can be performed to confirm the diagnosis.


Asunto(s)
Linfadenitis Necrotizante Histiocítica , Piel/patología , Adulto , Antígenos CD , Diagnóstico Diferencial , Femenino , Linfadenitis Necrotizante Histiocítica/diagnóstico , Linfadenitis Necrotizante Histiocítica/patología , Linfadenitis Necrotizante Histiocítica/fisiopatología , Humanos , Inmunohistoquímica , Lactante , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/patología , Lupus Eritematoso Sistémico/fisiopatología , Linfadenitis/diagnóstico , Linfadenitis/patología , Linfadenitis/fisiopatología , Masculino
15.
Clin Microbiol Infect ; 9(7): 625-31, 2003 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-12925102

RESUMEN

OBJECTIVE: To study the serologic profile of several types of test for toxoplasmosis, in order to contribute to the interpretation of antibody kinetics. METHODS: The clinical and serologic features of 120 cases of lymphadenopathy with known time of clinical onset were studied during 18 months postinfection. Antibody kinetics was determined by Sabin-Feldman dye test, complement fixation with light antigen, IgM immunofluorescent antibody test, and IgM immunosorbent agglutination assay (IgM-ISAGA). Cell-mediated immunity was evaluated by the toxoplasmin skin test. RESULTS: Seventy-five female patients aged 11-54 years (median 27 years) and 45 male patients aged 3-59 years (median 17 years) were studied, 85% of whom were under 30 years of age. Cervical lymph nodes were involved throughout, generally on both sides, with more than one affected ganglion group in 88%. The predominant symptom was asthenia (69%), which persisted in some cases for several months. A negative Sabin-Feldman dye test in a lymphadenopathy with more than three weeks' evolution excludes a toxoplasma etiology. A positive Sabin-Feldman dye test with negative IgM-ISAGA almost invariably excludes recent infection. The Sabin-Feldman dye test was positive in 94% of patients with titers higher than 1 : 16 000 within the first three months. The IgM-ISAGA yielded 98% of positive results, of which 94% were high titers. Titers >/= 1 : 160 in the IgM immunofluorescent antibody test and complement fixation were found to be highly indicative of recent infection, since 87% and 91%, respectively, were found within the first three months. A negative skin test plus positive serology values indicates recent infection. CONCLUSION: Our results indicate that estimation of time of infection on the basis of serologic results is improved by the simultaneous application of several tests, and correlates closely with the presence of clinical lymphadenitis.


Asunto(s)
Linfadenitis/inmunología , Linfadenitis/fisiopatología , Toxoplasmosis/inmunología , Toxoplasmosis/fisiopatología , Adolescente , Adulto , Factores de Edad , Niño , Preescolar , Pruebas de Fijación del Complemento , Femenino , Técnica del Anticuerpo Fluorescente , Humanos , Sueros Inmunes/inmunología , Inmunoglobulina M/inmunología , Inmunoglobulina M/metabolismo , Cinética , Linfadenitis/diagnóstico , Masculino , Persona de Mediana Edad , Toxoplasmosis/diagnóstico
16.
Toxicology ; 121(2): 165-78, 1997 Aug 15.
Artículo en Inglés | MEDLINE | ID: mdl-9230448

RESUMEN

The possible adjuvant effect of diesel exhaust particles (DEP) on the response to the model allergen ovalbumin (OA) was studied in BALB/c mice using the popliteal lymph node (PLN) assay. In addition to changes in PLN weight, cell numbers and cell proliferation, specific serum IgE anti-OA antibody levels were measured. OA inoculated together with DEP into one hind footpad gave a significantly augmented response (increase in weight, cell numbers and cell proliferation) in the draining popliteal lymph node as compared to DEP or OA alone. Also, the local lymph node response was of longer duration when DEP were given with the allergen. Experiments in thymus-deficient nu/nu mice indicated that the lymph node response observed in BALB/c mice was of a specific immunologic character and not an unspecific inflammatory reaction. The OA-specific IgE response was increased in mice receiving OA together with DEP as compared to the response in mice receiving OA without DEP. Carbon black (CB) was given with and without OA in some experiments, as a surrogate for the non-extractable core of DEP. CB was found to resemble DEP in its capacity to increase the local lymph node response and serum specific IgE response to OA, but CB appeared to be slightly less potent than DEP. Thus, both DEP and CB had a significant adjuvant effect on the local immune-mediated inflammatory response and on the systemic specific IgE response to allergen. The results indicate that the non-extractable particle core contributes substantially to the adjuvant activity of DEP.


Asunto(s)
Carbono/toxicidad , Inmunoglobulina E/biosíntesis , Ganglios Linfáticos/efectos de los fármacos , Ovalbúmina/inmunología , Emisiones de Vehículos/toxicidad , Adyuvantes Inmunológicos/toxicidad , Análisis de Varianza , Animales , Carbono/inmunología , División Celular/efectos de los fármacos , Modelos Animales de Enfermedad , Sinergismo Farmacológico , Femenino , Miembro Posterior , Inmunoglobulina E/sangre , Inmunoglobulina G/biosíntesis , Inmunoglobulina G/sangre , Recuento de Leucocitos/efectos de los fármacos , Ganglios Linfáticos/inmunología , Ganglios Linfáticos/patología , Linfadenitis/inducido químicamente , Linfadenitis/fisiopatología , Ratones , Ratones Endogámicos BALB C , Ratones Desnudos , Tamaño de los Órganos/efectos de los fármacos , Ovalbúmina/toxicidad , Tamaño de la Partícula , Distribución Aleatoria , Organismos Libres de Patógenos Específicos
17.
Am J Med Sci ; 310(1): 31-3, 1995 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-7604837

RESUMEN

Most patients with Rhodococcus equi infection are immunocompromised by either HIV infection, malignancy, or medication. Diagnosis is frequently missed or delayed because the organisms, resembling diphtheroids on smears, may be regarded as contaminants. Their clinical, pathologic, histochemical, and microbiologic resemblance to mycobacteria can result in misdiagnosis. Two cases were seen recently in our institution. R. equi pericarditis developed in a 29-year-old woman with failed renal transplant and R. equi axillary lymphadenitis developed in an asymptomatic 27-year-old man. These patients are important because the former is the first reported case of R. equi pericarditis, and the second case was unusual because of the absence of immunocompromise.


Asunto(s)
Infecciones por Actinomycetales/complicaciones , Linfadenitis/etiología , Pericarditis/etiología , Rhodococcus equi/aislamiento & purificación , Adulto , Femenino , Humanos , Linfadenitis/fisiopatología , Masculino , Pericarditis/diagnóstico por imagen , Pericarditis/fisiopatología , Tomografía Computarizada por Rayos X
18.
Clin Rheumatol ; 15(1): 81-3, 1996 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-8929783

RESUMEN

The authors describe three cases of systemic lupus erythematosus (SLE) associated with Kikuchi's histiocitic necrotizing lymphadenitis (HNL). Two patients presented a cytomegalovirus infection concomitantly with Kikuchi's lymphadenitis; in one of them the onsets of SLE and HNL were simultaneous. In the third case an inguinal HNL was concomitant with vaginitis of unknown aetiology.


Asunto(s)
Infecciones por Citomegalovirus/complicaciones , Lupus Eritematoso Sistémico/complicaciones , Linfadenitis/complicaciones , Adulto , Infecciones por Citomegalovirus/diagnóstico , Infecciones por Citomegalovirus/fisiopatología , Diagnóstico Diferencial , Femenino , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/fisiopatología , Linfadenitis/patología , Linfadenitis/fisiopatología , Necrosis
19.
Emerg Med Clin North Am ; 5(2): 359-70, 1987 May.
Artículo en Inglés | MEDLINE | ID: mdl-3325278

RESUMEN

The emergency clinician will continue to be called on to evaluate inflammatory processes of the head and neck on a regular basis. We have attempted to describe the broad range of these problems, as well as our current considerations for diagnosis and management. Our goal has been to promote accurate diagnosis to allow early detection of situations calling for prompt medical or surgical intervention.


Asunto(s)
Antibacterianos/uso terapéutico , Enfermedades Linfáticas/terapia , Absceso Peritonsilar/terapia , Drenaje , Urgencias Médicas , Cabeza/anatomía & histología , Humanos , Linfadenitis/tratamiento farmacológico , Linfadenitis/etiología , Linfadenitis/fisiopatología , Enfermedades Linfáticas/etiología , Enfermedades Linfáticas/fisiopatología , Sistema Linfático/anatomía & histología , Cuello/anatomía & histología , Absceso Peritonsilar/fisiopatología
20.
Am J Vet Res ; 47(8): 1722-5, 1986 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-3752680

RESUMEN

The effect of age on susceptibility of young pigs to streptococcic lymphadenitis was investigated. Twenty-nine cesarean-derived, colostrum-deprived pigs were allotted to 7 groups exposed to type IV group E Streptococcus (GES) at 5, 14, 28, 35, 56, 70, and 84 days of age. Four cesarean-derived, colostrum-deprived pigs were maintained as nonexposed controls. Six naturally farrowed, susceptible controls were exposed to GES at 63 to 84 days of age. All exposed pigs were killed and necropsied 28 days after exposure. Lesions of streptococcic lymphadenitis were not observed in pigs exposed at 5 or 14 days of age, except for 1 microabscess in a mandibular lymph node in a pig exposed at 14 days, but GES was recovered from 11% of lymph nodes examined from pigs of those age groups. Lesions and GES-positive lymph nodes were frequent in cesarean-derived, colostrum-deprived pigs exposed at 28 days and older and in susceptible controls. Serologic response to exposure, as determined by microtitration agglutination test and bactericidal test, was observed only in pigs exposed at 14 days and older. The absence of abscess development in pigs exposed at 5 or 14 days of age was not caused by antibody or failure of infecting organisms to reach the target organs.


Asunto(s)
Calostro/fisiología , Linfadenitis/veterinaria , Infecciones Estreptocócicas/veterinaria , Enfermedades de los Porcinos/fisiopatología , Animales , Susceptibilidad a Enfermedades , Femenino , Ganglios Linfáticos/patología , Linfadenitis/microbiología , Linfadenitis/patología , Linfadenitis/fisiopatología , Masculino , Embarazo , Infecciones Estreptocócicas/patología , Infecciones Estreptocócicas/fisiopatología , Porcinos , Enfermedades de los Porcinos/microbiología
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