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1.
J Pediatr Adolesc Gynecol ; 33(4): 429-431, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32224248

RESUMO

BACKGROUND: Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome is a cyclic autoinflammatory disease generally diagnosed in childhood. There have been studies suggesting a relationship between menstruation and other autoinflammatory syndromes such as familial Mediterranean fever (FMF), but not PFAPA specifically. CASE: This case describes a patient with a diagnosis of PFAPA who experienced complete resolution with tonsillectomy only to have recurrence of symptoms with onset of menstruation. She experienced symptom control with initiation of oral contraceptives. SUMMARY AND CONCLUSION: Prior to this case report, there had been no evidence in the literature suggesting a relationship between PFAPA and menstruation despite the observed association in other autoinflammatory syndromes. Onset of menses may be a trigger in PFAPA.


Assuntos
Febre/complicações , Linfadenite/complicações , Menstruação/fisiologia , Faringite/complicações , Estomatite Aftosa/complicações , Criança , Feminino , Febre/diagnóstico , Humanos , Linfadenite/diagnóstico , Masculino , Faringite/diagnóstico , Recidiva , Estomatite Aftosa/diagnóstico , Síndrome
4.
Int J Pediatr Otorhinolaryngol ; 127: 109655, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31470203

RESUMO

INTRODUCTION: Paediatric acute cervical lymphadenitis is a frequent diagnosis in the emergency department. Traditionally, suppurative cervical lymphadenitis (SCL) is associated with a higher need of surgical drainage. However, a great variability in the management of this suppurative infections can be observed. Moreover, the clinical distinction between non-suppurative cervical lymphadenitis (NSCL) and SCL is not an easy task and there are, currently, no guidelines defining which patients are eligible for imaging study. OBJECTIVES: To assess the determinants and benefits in the surgical management of SCL in children. As secondary outcome, to determine differences in epidemiological characteristics, clinical, biochemical and radiological features between NSCL and SCL. MATERIAL AND METHODS: A retrospective survey was carried out in a tertiary university hospital between January 2007 and December 2016. Forty-two children with a diagnosis of acute cervical lymphadenitis (ACL) were included and categorized according to the presence of suppuration, resulting in two groups: NSCL and SCL. The latter group was further categorized into surgical and non-surgical groups, according to the need of surgical drainage. RESULTS: No significant differences were found between SCL and NSCL groups in gender, age and previous antibiotics intake (p > 0.05). According to clinical presentation, odynophagia was significantly more frequent in NSCL patients (p = 0.01), with no differences found in other clinical parameters (p > 0.05). Patients presenting acute cervical lymphadenitis involving the submandibular region have 16 times the odds of a suppurative process (p = 0.029). In a SCL subgroup analysis, no association was observed between lymphadenitis size or location and the need for surgical drainage (p > 0.05). Children included in the SCL surgical group presented a trend to an increased in the hospitalization length (p = 0.01), when comparing to those in which treatment was limited to intravenous antibiotics. One death was observed in the SCL non-surgical group. CONCLUSIONS: Predictive factors for the need of surgery were not found. Furthermore, surgical drainage was not associated with better outcomes. Surgery could be considered in selected stable patients, when alternative medical treatments do not seem to work, in a case-to-case basis.


Assuntos
Antibacterianos/uso terapêutico , Drenagem , Linfadenite/terapia , Doença Aguda , Pré-Escolar , Feminino , Humanos , Lactente , Tempo de Internação , Linfadenite/complicações , Linfadenite/diagnóstico por imagem , Masculino , Pescoço , Seleção de Pacientes , Estudos Retrospectivos , Supuração/diagnóstico por imagem , Supuração/etiologia , Supuração/terapia
5.
BMC Pediatr ; 19(1): 211, 2019 06 28.
Artigo em Inglês | MEDLINE | ID: mdl-31253125

RESUMO

BACKGROUND: Intussusception is a gastrointestinal condition in which early treatment is critical. Although its epidemiology and comorbidities have been studied, few studies have included the entire pediatric population of a country. Therefore, we aimed to analyze the epidemiologic features of pediatric intussusception patients and identify comorbidities associated with intussusception in South Korea, using the public health database. METHODS: We analyzed the data of children below 18 years of age, from the national database of South Korea, who were diagnosed with intussusception and managed such as air reduction or surgical methods from 2008 to 2016. Patients were categorized into six groups based on the comorbid diseases. Patients with structural lesion in gastrointestinal tract were divided diagnosis or diagnosis code. RESULTS: The number of patients diagnosed with intussusception were 25,023 (16,024 males, 64.0%). Of them, the highest percentage was patients aged between 2 and 36 months (20,703; 82.7%). The incidence per 100,000 individuals aged up to 2 years was 196.7. The number of males were 16,024 (64.0%) and were almost twice the number of 8999 (36.0%) female patients. The maximum number of cases (n = 2517; 10.1%) were seen in September, followed by July (n = 2469; 9.9%). In February, the number of cases was lowest at 1448 (5.8%) patients (P < 0.001). The number of patients with structural lesions of the gastrointestinal tract that could lead to intussusception was 1207 (4.8%), while patients with acute gastrointestinal infectious disease were 4541 (18.1%). Among the structural lesions of the gastrointestinal tract that could be the leading cause of intussusception, lymphadenopathy was the most common, seen in 462 (56.6%) patients and an appendix-related condition was seen in 260 (31.9%) patients. Infectious diseases were more common in the younger children, while systemic diseases were more common in the older. CONCLUSION: We confirmed that pediatric intussusception in South Korea shows a seasonal tendency, which is age-dependent and is associated with an exposure to infectious agents. Some infectious pathogens and underlying diseases might play an important role in the pathophysiology of intussusception.


Assuntos
Intussuscepção/epidemiologia , Adolescente , Distribuição por Idade , Criança , Pré-Escolar , Comorbidade , Feminino , Gastroenteropatias/complicações , Humanos , Incidência , Lactente , Recém-Nascido , Intussuscepção/etiologia , Linfadenite/complicações , Masculino , República da Coreia/epidemiologia , Estações do Ano , Distribuição por Sexo
6.
Int J Pediatr Otorhinolaryngol ; 123: 63-65, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31075708

RESUMO

We describe the case of a young boy with severe clinical symptoms which mimicked several findings consistent with a peritonsillar abscess (PTA). After the patient exhibited no improvement with medical management, imaging revealed an intramuscular medial pterygoid abscess secondary to parapharyngeal space lymphadenitis. This is the first reported case of a patient with a medial pterygoid abscess secondary to lymphadenitis. We also describe a minimally invasive surgical approach for incision and drainage of the abscess that has not previously been described in the literature.


Assuntos
Abscesso/diagnóstico por imagem , Abscesso/cirurgia , Linfadenite/diagnóstico , Músculos Pterigoides , Abscesso/etiologia , Criança , Drenagem , Humanos , Linfadenite/complicações , Masculino , Tomografia Computadorizada por Raios X
7.
Anaerobe ; 57: 115-116, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31002871

RESUMO

Eggerthia catenaformis is a Gram-positive anaerobic rod, which has been rarely reported in human diseases. We report the second case of bacteremia due to this microorganism in an elderly patient. A 73-year-old man, without underlying diseases presented with fever, odynophagia and swelling of the cervical lymph node for several days. Culture of drained cervical fluid resulted in the isolation of Raoultella ornithinolytica and Streptococcus anginosus. Anaerobic blood cultures yielded a rare anaerobic microorganism, identified as Eggerthia catenaformis. No resistance to tested antimicrobials was documented. Treatment with drainage and several antibiotic regimens was established, and the general condition of the patient improved, at two months of follow-up.


Assuntos
Abscesso/complicações , Bacteriemia/diagnóstico , Bacteriemia/patologia , Firmicutes/isolamento & purificação , Infecções por Bactérias Gram-Positivas/diagnóstico , Infecções por Bactérias Gram-Positivas/patologia , Doenças Periodontais/complicações , Abscesso/diagnóstico , Idoso , Antibacterianos/uso terapêutico , Sangue/microbiologia , Drenagem , Enterobacteriaceae/isolamento & purificação , Infecções por Enterobacteriaceae/diagnóstico , Infecções por Enterobacteriaceae/microbiologia , Humanos , Linfadenite/complicações , Linfadenite/diagnóstico , Linfadenite/microbiologia , Linfadenite/terapia , Masculino , Doenças Periodontais/diagnóstico , Infecções Estreptocócicas/diagnóstico , Infecções Estreptocócicas/microbiologia , Streptococcus anginosus/isolamento & purificação
8.
Acta Paediatr ; 108(8): 1385-1392, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30901126

RESUMO

AIM: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most common cause of a periodic fever in childhood. The exact pathogenesis and the aetiology of PFAPA are still unknown. METHODS: We conducted a non-systematic review of published articles about PFAPA syndrome and summarised the evidence for diagnostic criteria and treatment options for PFAPA. RESULTS: The first proposed diagnostic criteria for PFAPA, in addition to periodic fever, included aphthous stomatitis, pharyngitis or cervical lymphadenitis in children younger than five years at the beginning of the symptoms. C-reactive protein (CRP) levels and leucocyte counts increase in most patients during episodes. Recent research reveals that tonsillectomy provides an immediate and long-lasting cure for PFAPA, even in the absence of classic criteria of aphthous stomatitis, pharyngitis or cervical adenitis and in children older than five years. CONCLUSION: We suggest that PFAPA can be diagnosed in children with at least five regularly occurring fever episodes without any other explanation, even in the absence of aphthous stomatitis, pharyngitis or cervical lymphadenitis and also in children older than five years.


Assuntos
Febre/complicações , Linfadenite/complicações , Faringite/complicações , Estomatite Aftosa/complicações , Pré-Escolar , Diagnóstico Diferencial , Febre/diagnóstico , Humanos , Linfadenite/diagnóstico , Faringite/diagnóstico , Estomatite Aftosa/diagnóstico , Síndrome
10.
Rheumatol Int ; 39(6): 957-970, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30798384

RESUMO

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a recurrent fever syndrome of early childhood with increasing number of adult-onset cases. Although it is a self-limited disease, it may negatively affect the quality of life. The aim of this review is to present a detailed analysis of PFAPA syndrome and an algorithm for diagnosis, therapeutic options, and evaluation of outcome. A comprehensive literature search was conducted through the Cochrane Library, Scopus, and MEDLINE/PubMed databases. The main topics covered are the epidemiology, clinical manifestations, diagnosis, differential diagnosis, etiopathogenesis, genetics, management, disease course and prognosis, disease in adults, unsolved issues, and unmet needs in PFAPA. The diagnosis of PFAPA is mainly based on clinical classification criteria. The most relevant hypothesis for pathogenesis is that dysregulated immune system in a genetically predisposed individual responds to a yet unidentified trigger in an exaggerated way. The pedigree analyses suggest a genetic background for the disease with an autosomal dominant pattern of inheritance. For management, single-dose corticosteroids during attacks and tonsillectomy remain the most effective therapies, while colchicine is a promising option to decrease attack frequency. There remain unsolved issues in PFAPA such as the exact etiopathogenesis and genetic background, the reason why the inflammation is restricted to the oropharyngeal lymphoid tissue, reasons for clock-work regularity of attacks, and self-limited disease course. There is need for a valid diagnostic criteria set with a high performance for both children and adults and consensus on management of PFAPA.


Assuntos
Febre/imunologia , Inflamação/imunologia , Linfadenite/imunologia , Faringite/imunologia , Estomatite Aftosa/imunologia , Corticosteroides/uso terapêutico , Febre/complicações , Febre/diagnóstico , Febre/terapia , Humanos , Inflamação/diagnóstico , Inflamação/terapia , Linfadenite/complicações , Linfadenite/diagnóstico , Linfadenite/terapia , Pescoço , Faringite/complicações , Faringite/diagnóstico , Faringite/terapia , Recidiva , Estomatite Aftosa/complicações , Estomatite Aftosa/diagnóstico , Estomatite Aftosa/terapia , Síndrome , Tonsilectomia
11.
Retin Cases Brief Rep ; 13(4): 367-370, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-28570435

RESUMO

BACKGROUND/PURPOSE: To report two novel cases of intermediate uveitis associated with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis syndrome. METHODS: Observational case reports and review of the literature. RESULTS: Both patients in this report had an established diagnosis of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis syndrome before the onset of ocular inflammation. Infectious and noninfectious systemic conditions known to be associated with intermediate uveitis were excluded. Intermediate uveitis was confirmed clinically in both patients by the presence of vitritis, snowballs, and peripheral snowbanks in the region of the pars plana. Both cases had a course characterized by recurrent inflammation; in which systemic steroid treatment, and in one case, immunomodulatory therapy was necessary. CONCLUSION: Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis syndrome is an auto-inflammatory fever disorder in childhood. Although other auto-inflammatory disorders such as, Blau syndrome, Muckle-Wells syndrome, and Behcets disease have been associated with various forms of uveitis, Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis has never been reported to be associated with any type of ocular inflammation. We describe for the first time, two cases of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis syndrome presenting with intermediate uveitis.


Assuntos
Diabetes Mellitus Tipo 1/complicações , Febre/complicações , Linfadenite/complicações , Faringite/complicações , Estomatite Aftosa/complicações , Uveíte Intermediária/etiologia , Acuidade Visual , Criança , Diabetes Mellitus Tipo 1/imunologia , Febre/imunologia , Angiofluoresceinografia , Fundo de Olho , Humanos , Linfadenite/imunologia , Masculino , Pescoço , Faringite/imunologia , Estomatite Aftosa/imunologia , Síndrome , Tomografia de Coerência Óptica/métodos , Uveíte Intermediária/diagnóstico
13.
World J Urol ; 37(4): 701-708, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30046844

RESUMO

PURPOSE: A significant proportion of patients affected by renal cell carcinoma (RCC) shows a suspicious lymph node involvement (LNI) at preoperative imaging. We sought to evaluate the effect of lymphadenopathies (cN1) on survival in surgical RCC patients with no evidence of LNI at final pathology (pN0). METHODS: 719 patients underwent either radical or partial nephrectomy and lymph node dissection at a single tertiary care referral centre between 1987 and 2015. All patients had pathologically no LNI (pN0). Outcomes of the study were cancer-specific mortality (CSM) and other-cause mortality. Multivariable competing-risks regression models assessed the impact of inflammatory lymphadenopathies (cN1pN0) on mortality rates, after adjustment for clinical and pathological confounders. RESULTS: 114 (16%) and 605 (84%) patients (16%) were cN1pN0 and cN0pN0, respectively. cN1pN0 patients were more frequently diagnosed with larger tumours (8.4 vs. 6.5 cm), higher pathological tumour stage (pT3-4 in 71 vs. 36%), higher Fuhrman grade (G3-G4 in 64 vs. 31%), more frequently with necrosis (75 vs. 44%), and distant metastases (33 vs. 10%) (all p < 0.0001). At univariable analysis, inflammatory lymphadenopathies resulted associated with worse CSM (HR 2.45; p < 0.0001). However, at multivariable analysis, inflammatory lymphadenopathies were not an independent predictor of CSM (HR 0.81; p = 0.4). The presence of metastases at diagnosis was the most important factor affecting CSM (HR 6.54; p < 0.0001). This study is limited by its retrospective nature. CONCLUSIONS: In RCC patients, inflammatory lymphadenopathies (cN1pN0) are associated with unfavourable clinical and pathological characteristics. However, the presence of inflammatory lymphadenopathies does not affect RCC-specific mortality.


Assuntos
Carcinoma de Células Renais/cirurgia , Neoplasias Renais/cirurgia , Excisão de Linfonodo , Linfadenite/patologia , Idoso , Carcinoma de Células Renais/complicações , Carcinoma de Células Renais/mortalidade , Carcinoma de Células Renais/patologia , Causas de Morte , Feminino , Humanos , Inflamação , Neoplasias Renais/complicações , Neoplasias Renais/mortalidade , Neoplasias Renais/patologia , Linfadenite/complicações , Linfadenopatia/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Mortalidade , Nefrectomia , Prognóstico
14.
Tuberk Toraks ; 66(3): 212-216, 2018 Sep.
Artigo em Turco | MEDLINE | ID: mdl-30479228

RESUMO

Introduction: Granulomatous lung disease (GLD) is caused by a wide range of conditions and it is challenge for pulmonologist. A detailed history of exposures is fundamental in GDL and has been found pivotal to reach a precise diagnosis. Materials and Methods: Between September 2014 and December 2016, the distribution of patients diagnosed with granulomatous lymphadenitis in the mediastinal/hilar lymph nodes by endobronchial ultrasound (EBUS) or mediastinoscopy was analyzed. To be listed as 'confident', a diagnosis of sarcoidosis required compatible histological, radiological and clinical findings in conjunction with negative cultures. Infectious entities listed as 'confident' had either microorganisms in tissue section, positive culture, positive serology or positive antigen detection in a consistent clinical pathological setting. Result: Granulomatous lymphadenitis was detected in 110 patients. The included 110 cases consisted of 70.9% women and median age of 53 (range 44-61) years. The final diagnosis of the patients was accepted to be sarcoidosis in 79 (71.8%), sarcoid like granulomas in 7 (6.4%), tuberculosis in 4 (3.6%), silicosis in 4 (3.6%), drug-associated granuloma in 2 (1.8%), hypersensitivity pneumonitis in 1 (0.9%), Chron disease in 1 (0.9%), unspecified in 12 (10.9%). Three patients were classified as tuberculosis based on culture. Conclusions: In this study, we found that the most common cause of granulomatous lymphadenitis was sarcoidosis. Contrary to expectations, the number of patients diagnosed with tuberculosis was very low.


Assuntos
Granuloma/patologia , Linfadenite/patologia , Mediastino/patologia , Adulto , Feminino , Granuloma/complicações , Granuloma/diagnóstico por imagem , Humanos , Linfonodos/patologia , Linfadenite/complicações , Linfadenite/diagnóstico por imagem , Masculino , Mediastino/diagnóstico por imagem , Pessoa de Meia-Idade , Ultrassonografia
15.
J Med Case Rep ; 12(1): 299, 2018 Oct 15.
Artigo em Inglês | MEDLINE | ID: mdl-30318016

RESUMO

BACKGROUND: Lymphadenopathy is not an uncommon presentation of adult onset Still's disease: it is present in up to two thirds of patients with adult onset Still's disease. The characteristic appearance of lymphadenopathy is described as intense, paracortical immunoblastic hyperplasia. Changes in light microscopy may resemble lymphoma, but immunohistochemistry reveals a benign polyclonal B cell hyperplasia. CASE PRESENTATION: We describe a 67-year-old Sri Lankan woman who manifested relapsing prolonged fever, raised inflammatory markers, arthralgia, myalgia, transient skin rash, and cervical lymphadenopathy histologically characterized by noncaseating granulomatous adenitis with central suppuration. Due to the fact of high prevalence of tuberculosis in the region, an extensive diagnostic evaluation was done to exclude the possibility of extrapulmonary tuberculosis; unsuccessful therapeutic trials of complete antituberculosis regime reliably excluded the possibility of tuberculosis and strengthened the diagnostic validity. Disease flares were characterized by systemic inflammatory response syndrome with immediate clinical and laboratory response to corticosteroids. After systematic diagnostic workup which ruled out possible malignant, rheumatic, or autoimmune diseases and infections previously described as causes of granulomatous adenitis, our patient was diagnosed as having adult onset Still's disease based on Yamaguchi criteria. She required a trial of indomethacin followed by methylprednisolone pulse therapy and long-term maintenance steroid therapy without steroid-sparing immunosuppressive agents or biological disease-modifying antirheumatic drugs. She achieved full disease remission in 3 months. Reevaluation after 6 months and 1 year did not reveal residual disease activity. CONCLUSIONS: To the best of our knowledge this is the first report of suppurative noncaseating granulomatous lymphadenitis attributed to adult onset Still's disease among Asian or South Asian ethnicities and it is also rarely reported among Europeans and North Americans. It is an extremely challenging situation to diagnose Still's disease with granulomatous lymphadenitis where tuberculosis is highly prevalent. This case highlights the importance of consideration of adult onset Still's disease as a potential diagnosis in a compatible clinical context in the presence of noncaseating granulomatous adenitis and indicates that one should not be misled into a diagnosis of tuberculosis by the fact of the high prevalence of tuberculosis, however, the exclusion of other diagnoses is a prerequisite.


Assuntos
Granuloma/complicações , Granuloma/patologia , Linfadenite/complicações , Linfadenite/patologia , Doença de Still de Início Tardio/complicações , Tuberculose , Idoso , Anti-Inflamatórios/uso terapêutico , Anti-Inflamatórios não Esteroides/uso terapêutico , Diagnóstico Diferencial , Feminino , Granuloma/tratamento farmacológico , Humanos , Indometacina/uso terapêutico , Linfadenite/tratamento farmacológico , Metilprednisolona/uso terapêutico , Supuração
16.
Cir. pediátr ; 31(4): 171-175, oct. 2018. tab
Artigo em Espanhol | IBECS | ID: ibc-172930

RESUMO

Objetivos: Describir nuestra experiencia en el manejo de la linfadenitis por micobacterias no tuberculosas (LMNT). Material y métodos: Análisis retrospectivo de 65 pacientes (26 varones/ 39 mujeres) intervenidos en nuestro centro de LMNT durante los años 2010-2017. Se analizaron datos demográficos, pruebas complementarias realizadas y datos de seguimiento. Resultados: La edad media fue de 31 meses (rango 7 meses-12 años). Para el diagnóstico la radiografía de tórax se realizó al 92,3% de pacientes, siendo en todos normal. El Mantoux fue positivo en 20,3%, dudoso en 12,5% y negativo en 67,1%. La PAAF preoperatoria fue diagnóstica (linfadenitis granulomatosa necrotizante) en 93,7% mientras que el cultivo para micobacterias fue positivo solo en 23,4%, siendo el Mycobacterium lentiflavum el patógeno más frecuentemente encontrado. El tiempo de espera preoperatorio fue de 2,5 meses con un porcentaje de fistulización previo a la cirugía de 7,7%. La estancia hospitalaria media fue 1,1 días y no hubo complicaciones perioperatorias. El tiempo medio de seguimiento fue 5,5 meses (rango 1-24 meses) observándose 19 casos de parálisis facial temporal, de los cuales 2 persistieron al cabo de 1 año. Conclusiones: La LMNT es una enfermedad cada vez más frecuente en nuestro medio. Es importante realizar un diagnóstico y tratamiento precoz, puesto que el tratamiento quirúrgico ha demostrado ser seguro y eficaz asociando baja tasa de complicaciones. Creemos que los protocolos actuales se deben revisar, debido a la escasa rentabilidad de algunas pruebas complementarias


Aim of study: to describe our experience in the management of non-tuberculosis mycobacterial lymphadenitits (NTML). Methods: Retrospective analysis of patients who underwent surgery for NTML in our centre during the period between 2010-2017. Demographic data, diagnostic tests, treatment and follow up information were recovered from medical records. Results: 65 patients (26 male/39 female) with a mean age of 31 months (range 7 months-12 years) were intervened during the period of study. As diagnostic tests, chest X-ray was performed in 92.3% of patients with normal result in all cases, Mantoux test was positive in 20.3%, inconclusive in 12.5% and negative in 67.1%. Preoperative fine needle aspiration was positive for NTML (granulomatous necrotizing lymphadenitis) in 93.7% whereas culture for mycobacteria was only positive in 23.4% of cases, being Mycobacterium lentiflavum the most frequent agent found. Mean preoperative waiting time was 2.5 months with 7.7% of fistulization previous to surgery. Mean hospital stay was 1,1 days and there were no intraoperative complications. Mean follow up time was 5.5 months (range 1-24 months) during which 19 cases of temporal facial paralysis were noted, among which only 2 persisted after one year. Conclusions: NTML is a disease with a growing incidence in our country. It is important to make an early diagnosis and surgical treatment in order to avoid complications, as surgery has demonstrated to be safe and effective, with a low rate of complications. We believe the actual protocols should be revised/checked due to low effectiveness of diagnostic tests


Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Linfadenite/complicações , Linfadenite/diagnóstico , Linfadenite/cirurgia , Micobactérias não Tuberculosas/isolamento & purificação , Micobactérias não Tuberculosas/patogenicidade , Linfadenite/etiologia , Infecções por Mycobacterium não Tuberculosas/microbiologia , Radiografia Torácica/métodos , Estudos Retrospectivos , Espanha/epidemiologia , Biópsia por Agulha Fina/métodos
17.
Pediatr Rheumatol Online J ; 16(1): 60, 2018 Sep 21.
Artigo em Inglês | MEDLINE | ID: mdl-30241480

RESUMO

BACKGROUND: Diagnosis of Periodic Fever, Aphthous stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) syndrome is currently based on the modified Marshall's criteria, but no validated evidence based classification criteria for PFAPA has been established so far. METHODS: A multistep process, based on the Delphi and Nominal Group Technique was conducted. After 2 rounds of e-mail Delphi survey involving 21 experts in autoinflammation we obtained a list of variables that were discussed in an International Consensus Conference. Variables reaching the 80% of consensus between participants were included in the new classification criteria. In the second phase the new classification criteria and the modified Marshall's criteria were applied on a cohort of 80 pediatric PFAPA patients to compare their performance. RESULTS: The Delphi Survey was sent to 22 participants, 21 accepted to participate. Thirty variables were obtained from the survey and have been discussed at the Consensus Conference. Through the Nominal Group Technique we obtained a new set of classification criteria. These criteria were more restrictive in respect to the modified Marshall's criteria when applied on our cohort of patients. CONCLUSION: Our work led us to identify a new set of classification criteria for PFAPA syndrome, but they resulted to be too restrictive to be applied in daily clinical practice for the diagnosis of PFAPA.


Assuntos
Doenças Hereditárias Autoinflamatórias/diagnóstico , Consenso , Técnica Delfos , Diagnóstico Diferencial , Febre/complicações , Doenças Hereditárias Autoinflamatórias/classificação , Humanos , Linfadenite/complicações , Faringite/complicações , Estomatite Aftosa/complicações , Síndrome
18.
Orphanet J Rare Dis ; 13(1): 132, 2018 08 09.
Artigo em Inglês | MEDLINE | ID: mdl-30092788

RESUMO

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.


Assuntos
Linfadenite/fisiopatologia , Criança , Pré-Escolar , Febre Familiar do Mediterrâneo/complicações , Febre Familiar do Mediterrâneo/fisiopatologia , Feminino , Humanos , Linfadenite/complicações , Masculino , Faringite/complicações , Faringite/fisiopatologia , Psicometria , Qualidade de Vida , Índice de Gravidade de Doença
19.
BMC Pregnancy Childbirth ; 18(1): 207, 2018 Jun 04.
Artigo em Inglês | MEDLINE | ID: mdl-29866074

RESUMO

BACKGROUND: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome has been considered as a childhood syndrome. The underlying etiology of PFAPA syndrome is unclear however, currently considered as auto-immune inflammatory disease. Recently, a few cases of adult-onset of PFAPA syndrome have been reported. However, there is no report about the successful management of pregnancy complicated with PFAPA syndrome. CASE PRESENTATION: The patient was a 31-year-old woman who developed recurrent episodes of high fever associated with cervical adenitis, pharyngitis and vomiting started 9 months after a delivery. She was diagnosed with PFAPA syndrome and cimetidine 800 mg/day was initiated. Since then, these symptoms got better. Cimetidine treatment was discontinued since she became pregnant (6 weeks of pregnancy). Except one febrile episode at 8 weeks gestation, she did not develop a febrile episode during pregnancy. Peripheral blood Th1/Th2 ratio was decreased from the first trimester to the second trimester of pregnancy. Then again, the ratio was steadily elevated during the third trimester. At 38 weeks, she delivered a live born infant without any complication. Two months after delivery, she developed PFAPA syndrome again and cimetidine treatment was re-initiated. However, febrile episodes were not controlled well, and Th1/Th2 ratio was further elevated compared to pregnancy status. Colchicine 0.5 mg once a day was initiated. Symptoms were diminished and Th1/Th2 ratio was gradually decreased. CONCLUSION: There was no case report of pregnancy complicated with PFAPA syndrome, though there were several reports of adult-onset PFAPA cases without pregnancy. The current case may be the first case report of a successful pregnancy complicated with PFAPA. In this case, PFAPA symptoms were ameliorated during pregnancy, but reappeared after delivery. We speculate that PFAPA syndrome, a Th1 type immune disorder, might be improved due to the Th1 to Th2 shifting, which was induced by pregnancy. It is necessary to investigate further about PFAPA syndrome with pregnancy and Th1/Th2 immune responses in the future.


Assuntos
Doenças Hereditárias Autoinflamatórias/complicações , Linfadenite/complicações , Faringite/complicações , Complicações na Gravidez , Estomatite Aftosa/complicações , Doenças do Colo do Útero/complicações , Adulto , Feminino , Humanos , Linfadenite/congênito , Faringite/congênito , Gravidez , Estomatite Aftosa/congênito , Síndrome , Doenças do Colo do Útero/congênito
20.
Wiad Lek ; 71(3 pt 2): 746-750, 2018.
Artigo em Ucraniano | MEDLINE | ID: mdl-29783260

RESUMO

OBJECTIVE: Introduction: Insufficient basic knowledge on the mechanisms of the multifactoral etiology and pathogenesis of various forms of maxillofacial lymphadenitis of odontogenic and non-odontogenic nature in children causes difficulties in making differential diagnosis. The algorithm of their examination involves a large number of methods, each of which has its own advantages and disadvantages with variable informativeness, depending on the particular situation. The aim: The paper is aimed atfamiliarization of broad medical public with informativeness of diagnostic measures in the nonspecific and specific affection of lymph nodes of the maxillofacial area in children. PATIENTS AND METHODS: Materials and methods: A thorough comprehensive analysis and generalization of scientific achievements elucidated in the fundamental and periodical publications, relating to diseases of the lymphatic system, has been carried out. RESULTS: Results: It has been establishedthat, despite a large variety of diseases accompanied by the reaction of the lymph nodes of different anatomic localization, current diagnostic possibilities are potent to establish a clinical diagnosis in most cases. In this way, the current diagnostic model requires the interaction of clinicians, infectiologists, molecular biologists, geneticists and morphologists. In this regard, the issues of efficient organization of the diagnostic process, detailing all stages of the search for accurate diagnosis, are crucial. CONCLUSION: Conclusions: The collected material on various forms of lymphadenitis and their secondary affection is fragmentary to date due to the absence of the unified methodological approach to carrying out differential diagnosis, which requires generalization and systematization of scientific groundwork. Unfortunately, the algorithms of examination of this category of patients, especially with lymphadenopathy, are not sufficiently developed to date, indicating the need for further search and optimization of diagnostic criteria taking into account modern realities.


Assuntos
Linfadenite/diagnóstico , Cistos não Odontogênicos/diagnóstico , Cistos Odontogênicos/diagnóstico , Criança , Pré-Escolar , Diagnóstico Diferencial , Humanos , Linfadenite/complicações , Cistos não Odontogênicos/complicações , Cistos Odontogênicos/complicações
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