INTRODUCTION: Protracted febrile myalgia syndrome (PFMS) is a rare manifestation of familial Mediterranean fever (FMF), characterized by myalgia, fever and elevated inflammatory markers lasting several weeks. As the hallmark of FMF are short episodes of disease symptoms, the long duration of PFMS may lead to a delayed diagnosis and treatment. OBJECTIVES: 1. To perform a review of literature and rheumatology textbooks focused on clinical features and treatment of PFMS in children. 2. To present our own case. METHODS: All articles in Pub Med generated using the keywords "protracted febrile myalgia" and information on PFMS in seven rheumatology textbooks were collected. The systematic review was supplemented with our own case presentation. RESULTS: In total, 18 articles with 78 pediatric patients (including our own) were retrieved. More than half of the patients presented with PFMS as the first manifestation of FMF. All complained of myalgia, 65% of abdominal pain and 26% had a rash. Corticosteroids (CS) were effective in 77%. In all CS-refractory cases, anakinra was shown efficient. MRI was used in 5 patients and showed myositis in all of them. The scrutiny of seven rheumatology textbooks showed that PFMS presenting with myalgia was mentioned in six. Possible accompanying symptoms were described only once, the long duration of symptoms twice, the efficacy of corticosteroids three times and anakinra only once. The presented 6 year old patient manifested with fever, myalgia, abdominal pain and petechial rash lasting 6 weeks. She had undergone multiple diagnostic procedures before her parents mentioned a positive family history for FMF. The subsequent genetic testing confirmed a homozygosity for M694V pathogenic variant in the MEFV gene. CONCLUSION: The long duration of PFMS may be misleading to clinicians especially if PFMS occurs at manifestation of FMF. The fact that more than half of the reported patients experienced PFMS as the presenting symptom of FMF is one of the key findings of our study. Our case presentation demonstrates the importance of genetic testing early in suspected autoinflammatory diseases. Furthermore, MRI may be an important diagnostic tool showing myositis in PFMS.
- MeSH
- dítě MeSH
- familiární středomořská horečka * komplikace diagnóza MeSH
- horečka * diagnóza etiologie MeSH
- lidé MeSH
- magnetická rezonanční tomografie metody MeSH
- myalgie * diagnóza etiologie MeSH
- syndrom MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- systematický přehled MeSH
V této kazuistice se budeme věnovat případu 14letého chlapce, který byl přivezen zdravotnickou záchrannou službou (ZZS) na dětské oddělení naší nemocnice pro dva dny trvající horečky, dehydrataci a celkové vyčerpání. Kromě horeček se u chlapce za hospitalizace postupně rozvíjely příznaky respiračního infektu, objevily se průjmovité stolice a makulózní exantém s bílým dermografismem. Z anamnézy bylo při příjmu zjištěno, že se chlapec před dvěma dny vrátil z pobytu v tropické oblasti.
In this article we present a case of a fourteen-year-old boy who was transported via ambulance to our Pediatric Ward due to a two-day-lasting fever, dehydration and total exhaustion. Additionally, the patient progressively developed respiratory symptoms, diarrhoea and rash with white dermographism during hospitalization. The medical history revealed that the boy had been visiting a tropical destination two days prior to hospital admission.
- MeSH
- cestovní nemoci * MeSH
- dengue diagnóza přenos virologie MeSH
- diferenciální diagnóza MeSH
- horečka * diagnóza etiologie klasifikace mikrobiologie MeSH
- koinfekce etiologie klasifikace mikrobiologie MeSH
- lidé MeSH
- mladiství MeSH
- průjem diagnóza etiologie mikrobiologie MeSH
- Salmonella enterica klasifikace patogenita MeSH
- tracheitida diagnóza etiologie farmakoterapie mikrobiologie MeSH
- tropické lékařství MeSH
- Check Tag
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- Publikační typ
- přehledy MeSH
BACKGROUND: Systemic autoinflammatory disorders (SAIDs) represent a growing spectrum of diseases characterized by dysregulation of the innate immune system. The most common pediatric autoinflammatory fever syndrome, Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA), has well defined clinical diagnostic criteria, but there is a subset of patients who do not meet these criteria and are classified as undefined autoinflammatory diseases (uAID). This project, endorsed by PRES, supported by the EMERGE fellowship program, aimed to analyze the evolution of symptoms in recurrent fevers without molecular diagnosis in the context of undifferentiated AIDs, focusing on PFAPA and syndrome of undifferentiated recurrent fever (SURF), using data from European AID registries. METHODS: Data of patients with PFAPA, SURF and uSAID were collected from 3 registries including detailed epidemiological, demographic and clinical data, results of the genetic testing and additional laboratory investigations with retrospective application of the modified Marshall and PRINTO/Eurofever classification criteria on the cohort of PFAPA patients and preliminary SURF criteria on uSAID/SURF patients. RESULTS: Clinical presentation of PFAPA is variable and some patients did not fit the conventional PFAPA criteria and exhibit different symptoms. Some patients did not meet the criteria for either PFAPA or SURF, highlighting the heterogeneity within these groups. The study also explored potential overlaps between PFAPA and SURF/uAID, revealing that some patients exhibited symptoms characteristic of both conditions, emphasizing the need for more precise classification criteria. CONCLUSIONS: Patients with recurrent fevers without molecular diagnoses represent a clinically heterogeneous group. Improved classification criteria are needed for both PFAPA and SURF/uAID to accurately identify and manage these patients, ultimately improving clinical outcomes.
- MeSH
- aftózní stomatitida * diagnóza epidemiologie MeSH
- dědičné zánětlivé autoimunitní nemoci * diagnóza MeSH
- dítě MeSH
- faryngitida * diagnóza MeSH
- horečka etiologie diagnóza MeSH
- kojenec MeSH
- lidé MeSH
- lymfadenitida * diagnóza epidemiologie MeSH
- mladiství MeSH
- předškolní dítě MeSH
- recidiva MeSH
- registrace * MeSH
- retrospektivní studie MeSH
- Check Tag
- dítě MeSH
- kojenec MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Evropa MeSH
INTRODUCTION: Poor adoption of stroke guidelines is a problem internationally. The Quality in Acute Stroke Care (QASC) trial demonstrated significant reduction in death and disability with facilitated implementation of nurse-initiated. METHODS: This was a multi-country, multi-centre, pre-test/post-test study (2017-2021) comparing post implementation data with historically collected pre-implementation data. Hospital clinical champions, supported by the Angels Initiative conducted multidisciplinary workshops discussing pre-implementation medical record audit results, barriers and facilitators to FeSS Protocol implementation, developed action plans and provided education, with ongoing support co-ordinated remotely from Australia. Prospective audits were conducted 3-month after FeSS Protocol introduction. Pre-to-post analysis and country income classification comparisons were adjusted for clustering by hospital and country controlling for age/sex/stroke severity. RESULTS: Data from 64 hospitals in 17 countries (3464 patients pre-implementation and 3257 patients post-implementation) showed improvement pre-to-post implementation in measurement recording of all three FeSS components, all p < 0.0001: fever elements (pre: 17%, post: 51%; absolute difference 33%, 95% CI 30%, 37%); hyperglycaemia elements (pre: 18%, post: 52%; absolute difference 34%; 95% CI 31%, 36%); swallowing elements (pre: 39%, post: 67%; absolute difference 29%, 95% CI 26%, 31%) and thus in overall FeSS Protocol adherence (pre: 3.4%, post: 35%; absolute difference 33%, 95% CI 24%, 42%). In exploratory analysis of FeSS adherence by countries' economic status, high-income versus middle-income countries improved to a comparable extent. DISCUSSION AND CONCLUSION: Our collaboration resulted in successful rapid implementation and scale-up of FeSS Protocols into countries with vastly different healthcare systems.
- MeSH
- cévní mozková příhoda * diagnóza MeSH
- horečka diagnóza MeSH
- hyperglykemie * diagnóza MeSH
- lidé MeSH
- polykání MeSH
- poruchy polykání * diagnóza MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- Geografické názvy
- Austrálie MeSH
Autoinflamatorní onemocnění (autoinflammatory diseases, AID) tvoří skupinu vzácných zánětlivých nemocí, způsobených poruchou regulace mechanismů nespecifické imunity. Bez zjevné příčiny při nich dochází ke spontánním celkovým a/nebo lokálním projevům zánětlivé aktivity. Mezi typická autoinflamatorní onemocnění řadíme syndromy periodických horeček. Patří sem u nás nejčastěji se vyskytující periodická horečka asociovaná s aftózní stomatitidou, zánětem hltanu a mízních uzlin (syndrom PFAPA), u níž je příčina multifaktoriální a genetický podklad zatím nebyl definován, ale také klasické monogenní horečky, například syndrom hyper IgD nebo familiární středozemní horečka, s již objasněnými genetickými mutacemi. Podezření na geneticky vázané horečky vzniká při rodinném výskytu febrilních epizod, začátku potíží již v raném věku, ale také při opakujících se přidružených příznacích, například kožních, očních či kloubních. Hlavním rizikem neléčených monogenních periodických horeček je rozvoj sekundární amyloidózy. Vzniku orgánového poškození včetně ledvinného selhání může zabránit včasná protizánětlivá léčba.
Autoinflammatory diseases (AID) are a group of rare inflammatory diseases caused by the dysregulation of nonspecific immunity mechanisms. In the absence of an obvious cause, they result in spontaneous general and/or local manifestations of inflammatory activity. Typical autoinflammatory diseases include periodic fever syndromes. These include in our country prevailing periodic fever associated with aphthous stomatitis, pharyngitis and lymph node inflammation (PFAPA syndrome), in which the cause is multifactorial and the genetic cause has not yet been defined, as well as classic monogenic fevers, such as Hyper-IgD syndrome or Familial Mediterranean fever, with genetic mutations already known. Genetically linked fevers are suspected when there is a family history of febrile episodes, the onset of problems at an early age, but also when there are recurrent associated symptoms, such as skin, eye or joint symptoms. The main risk of untreated monogenic periodic fevers is the development of secondary amyloidosis. Early anti-inflammatory treatment can prevent the development of organ damage, including renal failure.
Increased temperature in humans is the symptom of many infectious diseases and it is thus an important diagnostic tool. Infrared temperature measurement methods have been developed and applied over long periods due to their advantage of non-contact and fast measurements. This study deals with a statistical evaluation of the possibilities and limitations of infrared/thermographic human temperature measurement. A short review of the use of infrared temperature measurement in medical applications is provided. Experiments and statistics-based evaluation to confirm the expected accuracy and limits of thermography-based human temperature measurement are introduced. The results presented in this study show that the standard deviation of the thermographic measurement of the eyes maximum temperature was 0.4-0.9 °C and the mean values differences from the armpit measurement were up to 0.5 °C, based on the used IR camera, even though near ideal measurement conditions and permanent blackbody correction were used. It was also shown that a certain number of outliers must be assumed in such measurements. Extended analyses including simulations of true negative/false positive, sensitivity/specificity and receiver operating characteristics (ROC) curves are presented. The statistical evaluation as well as the extended analyses show that maximum eyes temperature is more relevant than a forehead temperature examination.
- MeSH
- horečka diagnóza MeSH
- infračervené záření * MeSH
- lidé MeSH
- tělesná teplota MeSH
- teplota MeSH
- termografie * metody MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
Horečka jako symptom provází řadu infekčních i neinfekčních onemocnění. Některá jsou častá, jiná naopak velice raritní. V praxi se horečka běžně vyskytuje jako příznak virového nebo bakteriálního onemocnění, ale může signalizovat i jiná závažná onemocnění, nebo dokonce otravy či intoxikace. Následující článek by měl stručně a přehledně shrnovat základní management u pacienta s horečkou.
Fever is a symptom, which accompanies many infectious and non-infectious illnesses. Its causes may be various ranging from frequent to very rare. The most common causes are viral or bacterial infections, but fever can also be a sign of other serious diseases, even poisoning or drug intoxication. The following article should briefly summarize essential management of patient with fever.
- MeSH
- antipyretika aplikace a dávkování terapeutické užití MeSH
- diferenciální diagnóza MeSH
- dospělí MeSH
- horečka * diagnóza etiologie farmakoterapie MeSH
- lidé MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- Publikační typ
- přehledy MeSH
Main symptoms found in patients with same diseases as for example COVID-19 is febrile. The infrared thermography (IRT) represents a fast measurement in case of screening in public places. One of the limitations of IRT is the resolution of sensor, which has close connection with the distance between camera and ROI. To maximize the effectivity of resolution of the camera is to reduce the distance from the object. The aim of presented study showed the possibility how to protect the camera or medical staff that operates the device against potential infection or contamination from the person with infection. Two protective foils of different thickness (40 μm; 9 μm) were tested as a barrier between the IRT and the ROI (black body model and human face). Even though the results have shown that the transparent foils decrease linearly the measured value of the temperature, it can be used as a protective barrier between IRT and the object if an appropriate recalculation is done during analysis of IRT images. Results are acceptable in the case of 9μm foil especially. The authors see this possibility as a minor concession from IRT standards but as a great help in health protection. The transparent foil can be used as protective barrier of the infrared camera.
- MeSH
- COVID-19 prevence a kontrola MeSH
- horečka diagnóza MeSH
- infračervené záření MeSH
- kůže diagnostické zobrazování MeSH
- lidé MeSH
- tělesná teplota * MeSH
- termografie * metody MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- práce podpořená grantem MeSH
