ABSTRACT
OBJECTIVE: Cervicofacial lymphadenitis caused by non-tubercular mycobacterial (NTM) infections has the highest infection rate in children. Our objective was to assess patient demographics, treatment methods, and the impact of weather and geography on the incidence of disease in patients with NTM cervicofacial lymphadenitis. METHODS: The Pediatric Health Information System (PHIS) database was queried for data on all patients diagnosed with concurrent cervicofacial lymphadenopathy and NTM infection from 2004 to 2022. We assessed the association between weather patterns and NTM cervicofacial lymphadenitis by collecting monthly weather data from the NOAA National Center for Environmental Information. Incidence rates were calculated by dividing the number of cases by the total hospital discharges during the study period. RESULTS: Among 47 PHIS hospitals, there were 992 diagnoses of NTM cervicofacial lymphadenitis. The average age at diagnosis was 2 [IQR, 2-4], with 59 % female. Drainage of skin abscesses or lesions was performed for 93 (9.4 %) patients, while 15 (1.5 %) had an excisional procedure of the CPT codes assessed. The most common antibiotics utilized were cephalosporins (28 %), macrolides (27 %), and rifampin (12 %). The most common treatment method was surgery with antibiotics (37 %) followed by no treatment at all (35 %), surgery alone (17 %), and antibiotics alone (10 %). Of the 28 states included in the analysis, Washington (IR: 3.5) and Nebraska (IR: 3.3) had the highest incidence rates (IR) of NTM cervical lymphadenitis. The cases were relatively equally distributed across the different weather seasons within each U.S. geographic region. However, the overall average wind speed was weakly associated with increasing the risk of diagnosis when utilizing a mixed effect zero-inflated negative binomial model (Incidence Ratio: 1.07, 95 % CI: (1.01-1.14), p = 0.035). CONCLUSIONS: Our results indicate that the most common treatment method utilized in patients within our cohort with NTM cervicofacial lymphadenitis was the concurrent use of surgery and antibiotics. Our results also indicate there may be variation in the incidence rate among different states, but additional studies are needed as our cohort only included approximately 50 % of states within the U.S.
Subject(s)
Databases, Factual , Lymphadenitis , Mycobacterium Infections, Nontuberculous , Humans , Female , Mycobacterium Infections, Nontuberculous/epidemiology , Mycobacterium Infections, Nontuberculous/therapy , Mycobacterium Infections, Nontuberculous/diagnosis , Male , Lymphadenitis/epidemiology , Lymphadenitis/microbiology , Lymphadenitis/therapy , Child, Preschool , Incidence , United States/epidemiology , Anti-Bacterial Agents/therapeutic use , Neck/microbiology , Face , Child , Retrospective Studies , Nontuberculous Mycobacteria/isolation & purification , InfantABSTRACT
PURPOSE: We aim to compare the different treatment modalities of non-tuberculous cervicofacial lymphadenitis in children, by means of a retrospective study conducted in the University Hospitals of Leuven of patients treated between 2012 and 2022. METHODS: For this retrospective cohort study, data were collected and pseudonimised from 52 patients with non-tuberculous cervicofacial lymphadenitis, who were treated in our hospital between January 2012 and December 2022, either conservatively, antibiotically, surgically, or with a combination of these options. We only included patients who were considered immunocompetent. All of the included patients were below 10 years at time of treatment. We collected data regarding time to resolution and adverse effects, i.e., skin discoloration, excessive scar formation, fistula formation, persistence of adenopathies after treatment, need for additional treatment, facial nerve paresis/paralysis, or systemic side-effects due to antibiotic treatment. RESULTS: The mean time to resolution (in days) when looking at primary treatments, was shortest in partial excisions (16), followed by complete excisions (19), antibiotic therapy (129), incision and drainage (153), curettage (240), and finally conservative management (280). Taking into account isolated treatments (i.e., both primary and adjuvant), we also observed consistently faster time to resolution in surgical and antibiotic treatments when compared to conservative treatment. Antibiotic therapy (p = 0.003), incision and drainage (p = 0,004) were associated with a significantly higher need for adjuvant treatment. Curettage was associated with a higher incidence of fistula formation (p = 0,006) and higher number of adjuvant treatments (p = 0,002). CONCLUSIONS: This study shows a faster resolution of nontuberculous mycobacterial cervicofacial lymphadenitis in children when treated surgically, more specifically when treated with partial or complete lymph node excision. Antibiotic treatment also leads to faster resolution than conservative management. There was a low rate of complications, and no permanent facial nerve damage was reported.
Subject(s)
Facial Paralysis , Fistula , Lymphadenitis , Mycobacterium Infections, Nontuberculous , Child , Humans , Infant , Nontuberculous Mycobacteria , Retrospective Studies , Lymphadenitis/therapy , Lymphadenitis/epidemiology , Lymphadenitis/microbiology , Anti-Bacterial Agents/therapeutic use , Facial Paralysis/therapy , Facial Paralysis/drug therapy , Mycobacterium Infections, Nontuberculous/epidemiology , Mycobacterium Infections, Nontuberculous/surgeryABSTRACT
Due to its nonspecific clinical characteristics, histiocytic necrotizing lymphadenitis (HNL) is often misdiagnosed as a suppurative cervical lymphadenitis and lymphoma. Thus, this study aimed to investigate the clinical characteristics of HNL in pediatric patients. We retrospectively identified 61 patients with histopathologically confirmed HNL. Clinical and laboratory data, including age, sex, clinical manifestations, laboratory investigations, histological discoveries, treatment, and outcomes, were collected from the medical records to determine associations with extracervical lymph node (LN) involvement. The mean age of patients was 9.7 ± 2.8 years (range, 1.5-14.0 years), and the male-to-female ratio was 2.2:1. The most common systemic symptom was fever in all patients. The median pre-admission and total durations of fever were 13.0 (interquartile range [IQR]: 9.0-22.5 days) and 22.0 days (IQR: 17.0-33.0 days), respectively. Patients with temporary fever (< 2 weeks) had a higher peak temperature and were more likely to undergo LN biopsy after admission than those with a prolonged fever (≥ 2 weeks). Multivariate analysis revealed that peak temperature ≥ 40 °C was significantly associated with a longer fever duration (P = 0.023). Laboratory values showed leukopenia (68.9%), which presented more frequently in solitary cervical LNs than in extracervical LNs (82.4% vs. 52.9%, p = 0.027) in patients with prolonged fever. CONCLUSIONS: HNL is often misdiagnosed in older children with persistent fever and lymphadenopathy, leading to unnecessary diagnostic tests and evaluations, inappropriate antibiotic administration, and mismanagement. A multidisciplinary team, including primary care providers, rheumatologists, and pathologists, can improve patient outcomes by increasing their awareness of this rare condition. WHAT IS KNOWN: ⢠Histiocytic necrotizing lymphadenitis (HNL) is characterized by fever, leukopenia, and neck lymphadenopathy with unknown etiology. ⢠The lack of neutrophils or eosinophils in the histology, immunohistochemistry results help distinguish HNL from infectious causes. Although HNL is a self-limiting disease, antibiotics and steroid treatments were used inappropriately. WHAT IS NEW: ⢠A fever peak ≥ 40 °C was associated with a longer fever duration in HNL patients. Leukopenia presented more frequently in solitary cervical lymph node (LNs) than in extracervical LNs inpatients with prolonged fever. ⢠Steroids are not recommended as a routine treatment, however, in some severe or relapsing cases with persistent symptoms, prednisolone (5 mg twice a day for 2 days) or other steroids (an equivalent dose of prednisolone) responded favorably.
Subject(s)
Histiocytic Necrotizing Lymphadenitis , Leukopenia , Lymphadenitis , Lymphadenopathy , Humans , Male , Child , Female , Infant , Child, Preschool , Adolescent , Histiocytic Necrotizing Lymphadenitis/diagnosis , Histiocytic Necrotizing Lymphadenitis/therapy , Histiocytic Necrotizing Lymphadenitis/pathology , Retrospective Studies , Lymph Nodes/pathology , Lymphadenitis/diagnosis , Lymphadenitis/therapy , Lymphadenopathy/diagnosis , Lymphadenopathy/pathology , Fever , Prednisolone , Leukopenia/pathologyABSTRACT
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in pediatric patients. It is clinically characterized by fever flares lasting 3-7 days, reappearing every 2-8 weeks with a distinctive clockwork regularity. PFAPA generally begins before 5 years of age and usually ceases 3-5 years after onset. Recurrences may be observed in adolescence and adulthood in up to 20% of cases. The authors aim to describe a case of PFAPA recurrence in adolescence temporally associated with allergen-specific immunotherapy (ASIT). A 16-year-old female patient was referred to the rheumatology unit due to recurrent episodes of fever one month after initiating ASIT for allergic rhinitis. These episodes occurred every 4 weeks and lasted 3 days. During these episodes, she also presented with a sore throat, tonsillar exudates, and cervical lymphadenopathy. Abortive treatment with oral prednisolone was attempted in these episodes, with complete resolution of fever after a single dose. After reviewing her medical background, she had previously experienced febrile episodes accompanied by aphthous ulcers and tonsillar exudates occurring every 7-8 weeks from age 2-7. The etiopathogenesis of PFAPA remains uncertain. Environmental triggers, particularly those with immunomodulator effects, may interfere with the immune responses responsible for PFAPA occurrence, but the mechanisms are still unclear. The authors describe the first report of the reappearance of PFAPA flares, possibly due to ASIT. Further studies are needed to fully clarify if ASIT constitutes a true environmental trigger of PFAPA.
Subject(s)
Amyloidosis , Lymphadenitis , Lymphadenopathy , Pharyngitis , Stomatitis, Aphthous , Adolescent , Child, Preschool , Female , Humans , Allergens , Fever/etiology , Immunotherapy , Lymphadenitis/therapy , Lymphadenitis/complications , Pharyngitis/therapy , Pharyngitis/complications , Stomatitis, Aphthous/therapy , Stomatitis, Aphthous/complications , SyndromeABSTRACT
ABSTRACT: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is, as the name implies, characterized by an extremely regular cycle of fevers that is accompanied by one or more other symptoms such as oral ulcers, pharyngitis, adenitis, tonsillitis, sore throat, cervical adenopathy, and headache. Originally known as Marshall syndrome, PFAPA is most commonly identified in children younger than age 5 years; however, adults may also present with the disease, though they may report additional symptoms. PFAPA is now understood to be a diagnosis of exclusion. Laboratory studies are typically unremarkable except for increases in acute phase reactants such as C-reactive protein. Treatment is primarily supportive and most frequently uses systemic steroids to suppress the inflammatory response. Acute flares are self-limited, and the syndrome typically resolves on its own as the child reaches age 7 or 8 years.
Subject(s)
Lymphadenitis , Lymphadenopathy , Pharyngitis , Stomatitis, Aphthous , Adult , Child , Humans , Child, Preschool , Stomatitis, Aphthous/diagnosis , Stomatitis, Aphthous/therapy , Lymphadenitis/diagnosis , Lymphadenitis/therapy , Pharyngitis/diagnosis , Syndrome , Fever/etiologyABSTRACT
BACKGROUND: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an autoinflammatory disease occurring in children. Although PFAPA is the most common periodic fever syndrome found in children, there are only a few studies defining the clinical characteristics and the efficacy of treatment strategies among Japanese children. This study aimed to clarify the demographic characteristics and clinical features of patients with PFAPA syndrome and to evaluate treatment efficacy. METHODS: We retrospectively reviewed the clinical features of children with PFAPA who visited Saitama Children's Medical Center between January and December 2019. We also evaluated treatment strategies and their efficacy; abortive treatment with corticosteroids, prophylaxis with cimetidine or colchicine, and surgical management with tonsillectomy. RESULTS: A total of 100 Japanese children (61% male) with PFAPA were included. Median age of onset was 3 years, median duration of fever episodes was 5 days, and median interval between episodes was 4 weeks. The symptoms (frequencies) were pharyngitis (89%), exudate on tonsils (71%), cervical adenitis (50%), and aphthous stomatitis (49%). Approximately 37% of patients took prednisolone for aborting fever attacks, showing a 100% response; 93% were treated with cimetidine, showing an 79.6% response, and 18% were treated with colchicine, showing a 66.7% response. Only one patient underwent tonsillectomy. CONCLUSIONS: Among Japanese children with PFAPA, 28% of them were ≥5 years with a male predominance. Pharyngitis is the most frequent symptom associated with fever. Cimetidine is suitable for initial therapy because of its safety and efficacy.
Subject(s)
Lymphadenitis , Lymphadenopathy , Pharyngitis , Stomatitis, Aphthous , Tonsillectomy , Child , Cimetidine/therapeutic use , Colchicine/therapeutic use , Female , Fever/complications , Fever/etiology , Humans , Infant, Newborn , Japan/epidemiology , Lymphadenitis/diagnosis , Lymphadenitis/epidemiology , Lymphadenitis/therapy , Lymphadenopathy/complications , Male , Pharyngitis/complications , Pharyngitis/diagnosis , Pharyngitis/therapy , Prednisolone , Retrospective Studies , Stomatitis, Aphthous/diagnosis , Stomatitis, Aphthous/epidemiology , Stomatitis, Aphthous/therapy , Syndrome , Treatment OutcomeABSTRACT
OBJECTIVE: To describe clinical, diagnostic and therapeutic characteristics of the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. DATA SOURCE: Literature review in the PubMed database by using specific descriptors to identify all articles published in the English language in the last three years; 38 articles were found. After performing selection of titles and abstract analysis, 13 out of the 38 articles were fully read. Relevant studies found in the references of the reviewed articles were also included. DATA SYNTHESIS: The PFAPA syndrome (Periodic Fever, Aphthous Stomatitis, Pharyngitis and cervical Adenitis) is a medical condition grouped among the periodic fever syndromes. The etiology is uncertain, but possibly multifactorial, and its symptoms are accompanied by recurrent febrile episodes although weight and height development are preserved. It is a self-limiting disease of benign course with remission of two to three years without significant interference in the patient's overall development. Treatment consists of three pillars: interruption of febrile episodes, increase in the interval between episodes, and remission. CONCLUSIONS: Despite several attempts to establish more sensitive and specific criteria, the diagnosis of PFAPA syndrome is still clinical and reached by exclusion, based on the modified Marshall's criteria. The most common pharmacological options for treatment include prednisolone and betamethasone; colchicine may be used as prophylaxis, and surgical treatment with tonsillectomy can be considered in selected cases.
Subject(s)
Lymphadenitis , Pharyngitis , Stomatitis, Aphthous , Child , Fever/diagnosis , Fever/etiology , Humans , Lymphadenitis/complications , Lymphadenitis/diagnosis , Lymphadenitis/therapy , Pharyngitis/complications , Pharyngitis/diagnosis , Pharyngitis/therapy , Stomatitis, Aphthous/complications , Stomatitis, Aphthous/diagnosis , Stomatitis, Aphthous/therapy , SyndromeABSTRACT
INTRODUCTION: As a recurrent disease, periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by episodes of febrile attacks and is often prominent in children under five years of age. However, the etiology of this condition has not been fully understood yet. MATERIALS AND METHODS: The search in the extensive literature of peer-reviewed articles published from the inception to December 2021 was conducted to identify the relevant studies, using the electronic databases of MEDLINE/PubMed, Embase, Scopus, the Cochrane Library, and the Web of Science. RESULTS: The analysis of complex relationships indicates that inflammatory factors, such as various cytokines and acute-phase proteins (APPs), play leading roles in the pathogenesis of this disease. Accordingly, this article summarizes the current state of knowledge to explain the mechanisms involved in inflammatory responses among patients with PFAPA syndrome and investigate its role in the pathogenesis of this disease. Moreover, the possibilities for further implementation of new therapeutic strategies are pointed out. CONCLUSION: It is concluded that some pathophysiological processes are associated with immune dysregulation, which itself may be secondary to environmental factors, genetic background, and underlying diseases, including latent infections that multiply inflammatory mediators. elevated inflammatory markers similarly play a significant part in the clinical outcomes of this condition, whose pyrogenic nature is the reason for the development of episodes of febrile attacks in the population of patients suffering from PFAPA syndrome.
Subject(s)
Amyloidosis , Lymphadenitis , Pharyngitis , Stomatitis, Aphthous , Child , Child, Preschool , Fever/complications , Fever/therapy , Humans , Inflammation Mediators , Lymphadenitis/complications , Lymphadenitis/therapy , Pharyngitis/complications , Pharyngitis/therapy , Stomatitis, Aphthous/complications , Stomatitis, Aphthous/therapy , SyndromeABSTRACT
An MHC class II deficient 2-year-old boy presented with fever and an enlarging left neck mass 100 days post allogeneic haematopoietic stem cell transplant (HSCT). Fever persisted despite treatment with broad-spectrum ß-lactam antibiotics. His BCG vaccination site at presentation was quiescent. Ultrasound showed enlarged cervical lymph nodes. An incisional biopsy of the large nodal mass yielded acid-fast bacilli, identified as Mycobacterium bovis by genome sequencing. Treatment with rifampicin, isoniazid and pyridoxine was started. The mass suppurated (figure 1), before healing concurrently with T-lymphocyte reconstitution at approximately day 130 post-HSCT.
Subject(s)
Hematopoietic Stem Cell Transplantation , Immune Reconstitution , Lymphadenitis , BCG Vaccine/adverse effects , Child , Child, Preschool , Hematopoietic Stem Cell Transplantation/adverse effects , Humans , Infant, Newborn , Isoniazid/therapeutic use , Lymphadenitis/chemically induced , Lymphadenitis/therapy , Male , RifampinABSTRACT
O objetivo deste trabalho foi avaliar a eficácia do florfenicol na dose usualmente empregada em equinos de 22 mg/kg pelas vias intravenosa, intramuscular e oral para o tratamento de adenite equina por Streptococcus equi. subsp. equi, usando a modelagem farmacocinética/farmacodinâmica (PK/PD Pharmacokinetic/Pharmacodynamic) e a simulação de Monte Carlo. Foi realizada uma simulação de Monte Carlo a partir dos parâmetros PK, logo depois, efetuou-se a modelagem PK/PD para determinar as taxas de eficácia do antimicrobiano para o tratamento dessa infecção bacteriana, de acordo com o valor da concentração inibitória mínima (CIM), em um intervalo de CIM de 0,125 4 μg/mL. Pela via intravenosa, a probabilidade de erradicação bacteriana foi de 100% para CIM até 0,5 μg/mL e efeito bacteriostático com probabilidades de 99% e 80% para CIMs de 2 e 4 μg/mL, respectivamente. Já pelas vias intramuscular e oral a probabilidade de se atingir o índice de erradicação bacteriológica foi de 100% para CIM de até 0,5 μg/mL, contudo, atinge valores de 80% e 81%, respectivamente, para CIM de 1 μg/mL considerando o efeito bactericida (p<0,01). Portanto, através desse estudo é evidenciado a eficácia do florfenicol até a CIM de 0,5 μg/mL para as três vias de administração citadas, entretanto, para CIMs superiores a esse valor, é imprescindível o ajuste da dose farmacológica, evitando falhas na terapêutica e possível resistência microbiana.
The objective of this study was to evaluate the efficacy of florfenicol at the dose usually used in horses of 22 mg/kg by intravenous, intramuscular and oral routes for the treatment of equine adenitis caused by Streptococcus equi. subsp. equi, using Pharmacokinetic/Pharmacodynamic (PK/PD) modeling and Monte Carlo simulation. A Monte Carlo simulation was performed from the PK parameters, then PK/PD modeling was performed to determine the antimicrobial efficacy rates for the treatment of this bacterial infection, according to the minimum inhibitory concentration (MIC) value, in a MIC range of 0.125 - 4 μg/mL. Intravenously, the probability of bacterial eradication was 100% for MICs up to 0.5 μg/mL, and the bacteriostatic effect was 99% and 80% for MICs of 2 and 4 μg/mL, respectively. However, for the intramuscular and oral routes, the probability of reaching the bacteriologic eradication index was 100% for MICs of up to 0.5 μg/mL, however, it reaches values of 80% and 81%, respectively, for MICs of 1 μg/mL considering the bactericidal effect (p<0.01). Therefore, through this study the efficacy of florfenicol is evidenced up to the MIC of 0.5 μg/mL for the three routes of administration cited, however, for MICs higher than this value, it is essential to adjust the pharmacological dose, avoiding failures in therapy and possible microbial resistance.
Subject(s)
Animals , Horses/physiology , Horses/injuries , Pharmacokinetics , Lymphadenitis/therapy , Lymphadenitis/veterinary , Thiamphenicol/analogs & derivatives , Thiamphenicol/pharmacokinetics , Thiamphenicol/pharmacology , Streptococcus equiABSTRACT
BACKGROUND: Nontuberculous mycobacteria (NTM) is a common cause of lymphadenitis. A rise in incidence has been reported. Our main aim was to describe the clinical features, microbiological aspects and treatment of the disease. METHODS: We conducted a retrospective, monocentric study between January 2008 and December 2017 (University Hospital of Nantes). INCLUSION CRITERIA: age<18 years, 1 positive lymph node specimen with identification of the species in culture, head-and-neck localization. RESULTS: Forty-nine patients were enrolled from 2008 to 2017. Median age was 28 months (range: 6-141 months). Median time to confirmation of diagnosis was 2.1 months (range: 0.7-6 months). The sites encountered were mandibular (45%), cervical (33%), and parotid (16%). The main clinical signs were a tender nodule (70%), purplish nodule (59%) or painless nodule (83%), without fever (88%). The species identified were: Mycobacterium avium (n=26), M. lentiflavum (n=13), M. intracellulare (n=7), M. malmoense (n=2) and M. scrofulaceum (n=1). Antibiotic treatment was frequent (77% of cases). DISCUSSION: This study is the second largest French cohort of NTM lymphadenitis in children with microbiological confirmation. The most frequent presentation was a tender, purplish, and painless mandibular nodule. The predominant species was M. avium. M. lentiflavum, which emerged during our study, does not figure in any European studies before 2014 but appears in the most recent studies. The effects of discontinuation of mandatory BCG immunization in France in NMT is not statistically demonstrable here due to lack of relevant data prior to 2007. CONCLUSION: A possible diagnosis of NTM lymphadenitis should not be overlooked in children presenting painless, purplish, cervicofacial tumefaction.
Subject(s)
Lymphadenitis , Mycobacterium Infections, Nontuberculous , Adolescent , Child , Child, Preschool , Humans , Lymph Nodes , Lymphadenitis/diagnosis , Lymphadenitis/epidemiology , Lymphadenitis/therapy , Mycobacterium Infections, Nontuberculous/diagnosis , Mycobacterium Infections, Nontuberculous/drug therapy , Mycobacterium Infections, Nontuberculous/epidemiology , Nontuberculous Mycobacteria , Retrospective StudiesABSTRACT
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.
Subject(s)
Fever/therapy , Lymphadenitis/therapy , Pharyngitis/therapy , Stomatitis, Aphthous/therapy , Adrenal Cortex Hormones/therapeutic use , Advisory Committees , Antipyretics/therapeutic use , Child , Child, Preschool , Cimetidine/therapeutic use , Colchicine/therapeutic use , Fever/physiopathology , Histamine H2 Antagonists/therapeutic use , Humans , Lymphadenitis/physiopathology , Neck , Pharyngitis/physiopathology , Stomatitis, Aphthous/physiopathology , Syndrome , Tonsillectomy , Tubulin Modulators/therapeutic useABSTRACT
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by recurrent fever flares accompanied by symptoms and signs forming the disease acronym and alternating with asymptomatic periods. Despite the disease having a generally favorable outcome, with spontaneous remission after a few years, it does have a major impact on the quality of life of the child and his or her relatives. Beside symptomatic medications during fever flares, the most used treatment consists of a single dose of corticosteroids at flare onset to interrupt the attack; fever resolves usually within a few hours, but often with a shorter interval between the attacks. For these patients, colchicine has been shown to decrease the frequency of the flares. Other medications were also reported in case series of patients with PFAPA syndrome. These include the interleukin-1 blocker anakinra to treat flares, cimetidine (which showed no convincing effect), and other drugs with anecdotal use. The pediatrician faces a difficult question: should they wait for spontaneous disease resolution and only treat the flares medically, or should they propose tonsillectomy that may induce remission? Due to the lack of strong evidence, the answer will vary on an individual basis, depending on the impact on the patient's quality of life. The choice of the best therapeutic strategy will be based on the response to the flare-based therapy and on disease severity (quality of life, school performances); long-term data on the remission rate under the different therapeutic strategies are currently missing. More randomized controlled studies are needed to help the treating physician to choose the best therapy, as well as real-life long-term data to evaluate the long-term outcome of children with PFAPA syndrome.
Subject(s)
Fever/therapy , Lymphadenitis/therapy , Pharyngitis/therapy , Quality of Life/psychology , Stomatitis, Aphthous/therapy , Child, Preschool , Female , Humans , Male , SyndromeABSTRACT
OBJECTIVE: To evaluate the long-term treatment results of patients with PFAPA syndrome and to determine their need for tonsillectomy. MATERIALS AND METHODS: The clinical characteristics, treatments and long-term results of 16 patients admitted to the Pediatric and Otorhinolaryngology Clinic between 2015 and 2019 were retrospectively analyzed. RESULTS: Twelve male and four female patients were examined between 1.5 and 8 years (mean age 4.8 ± 1.1) (75% male, 25% female). The mean duration of attacks was 4.4 ± 1.4 weeks. Twelve patients recovered completely with single-dose steroid therapy. In the 4-year follow-up of 12 patients who were given a single dose of steroid therapy, there were no relapses. Surgical decisions were made for four patients whose attacks did not pass with steroid treatment. Two patients (75%) underwent adenotonsillectomy, and one patient underwent tonsillectomy While three of these patients did not have an attack again, one patient continued to have an attack every 8 weeks. At the age of 9, his attacks were completely resolved spontaneously. All patients had fever. The most common concomitant symptoms were pharyngitis (94%), cervical adenitis (82%) and aphthous stomatitis (77%). Exudative tonsillitis was present in 25% of the patients. CONCLUSION: Medical treatment should be the first treatment option in patients with PFAPA syndrome. Although single-dose steroid treatment is effective in these patients, tonsillectomy is an important treatment option in refractory cases.
Subject(s)
Lymphadenitis , Pharyngitis , Stomatitis, Aphthous , Tonsillectomy , Adenoidectomy , Child , Child, Preschool , Female , Humans , Lymphadenitis/diagnosis , Lymphadenitis/therapy , Male , Pharyngitis/therapy , Retrospective Studies , Stomatitis, Aphthous/surgery , Stomatitis, Aphthous/therapySubject(s)
Aortitis/microbiology , Brucella melitensis/isolation & purification , Brucellosis/complications , Lymphadenitis/microbiology , Aged , Aneurysm, Ruptured/microbiology , Aneurysm, Ruptured/surgery , Aortic Aneurysm, Abdominal/microbiology , Aortic Aneurysm, Abdominal/surgery , Aortitis/therapy , Brucellosis/therapy , Cheese/microbiology , Humans , Lymphadenitis/therapy , MaleSubject(s)
Lymphadenitis/therapy , Mycobacterium Infections, Nontuberculous/therapy , Nontuberculous Mycobacteria , Skin Diseases, Bacterial/therapy , Anti-Bacterial Agents/therapeutic use , Child , Child, Preschool , Disease Management , Face/microbiology , Female , Humans , Infant , Lymphadenitis/microbiology , Male , Neck/microbiology , Skin Diseases, Bacterial/microbiologyABSTRACT
OBJECTIVE: Cervicofacial lymphadenitis caused by nontuberculous mycobacteria (NTM) is commonly treated with surgery or antimicrobial therapy. The aim of this study was to analyze the utility of our new blood-based diagnostic method and the treatment protocol, surgery or observation alone, in NTM lymphadenitis in children. METHODS: All patients under 16 years of age with cervicofacial NTM lymphadenitis diagnosed and treated at Children's Hospital or at the Department of Otorhinolaryngology, Helsinki University Hospital (Helsinki, Finland) in 2007-2017 were retrospectively reviewed. RESULTS: Fifty-two patients, 33 (63%) of whom were girls, were included in the study. The median age at initial presentation of the NTM lymphadenitis was 2.9 years. The novel blood-test had been performed on 49 (94%) of the patients and in all of them it was indicative of NTM infection. A sample for mycobacterial culture was available from 34 patients, and Mycobacterium avium was the most common species detected. Most patients (nâ¯=â¯33, 63%) were treated conservatively with observation alone. Of these, nine patients (27%) did not develop a skin fistula, and the lymphadenitis resolved without drainage. CONCLUSIONS: The novel blood test is clinically feasible method for diagnosing childhood cervicofacial NTM lymphadenitis noninvasively. Observation alone is a good alternative to surgery, without the risk of complications.
Subject(s)
Lymphadenitis/therapy , Mycobacterium Infections, Nontuberculous/diagnosis , Mycobacterium Infections, Nontuberculous/therapy , Watchful Waiting , Child , Child, Preschool , Drainage , Face , Female , Humans , Infant , Lymphadenitis/microbiology , Male , Mycobacterium Infections, Nontuberculous/blood , Mycobacterium Infections, Nontuberculous/complications , Neck , Retrospective StudiesSubject(s)
Lymphadenitis/diagnostic imaging , Mediastinal Diseases/diagnostic imaging , Nasopharyngeal Neoplasms/diagnostic imaging , Positron Emission Tomography Computed Tomography/methods , Tuberculosis, Lymph Node/diagnostic imaging , Fluorodeoxyglucose F18 , Humans , Lymphadenitis/complications , Lymphadenitis/therapy , Mediastinal Diseases/complications , Mediastinal Diseases/therapy , Mediastinum , Nasopharyngeal Neoplasms/complications , Nasopharyngeal Neoplasms/therapy , Positron-Emission Tomography , Treatment Outcome , Tuberculosis, Lymph Node/complications , Tuberculosis, Lymph Node/therapyABSTRACT
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 (pâ¯>â¯0.05). According to clinical presentation, odynophagia was significantly more frequent in NSCL patients (pâ¯=â¯0.01), with no differences found in other clinical parameters (pâ¯>â¯0.05). Patients presenting acute cervical lymphadenitis involving the submandibular region have 16 times the odds of a suppurative process (pâ¯=â¯0.029). In a SCL subgroup analysis, no association was observed between lymphadenitis size or location and the need for surgical drainage (pâ¯>â¯0.05). Children included in the SCL surgical group presented a trend to an increased in the hospitalization length (pâ¯=â¯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.