When, how, and why do fevers hold children hostage?

Rigante, Donato

doi:10.1111/jebm.12377

Cited by 13 publications

(5 citation statements)

References 43 publications

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“…Great progress has been achieved in the recognition of non-infectious, non-rheumatic or non-tumoral causes of recurrent fever, and dysregulated inflammasome activity caused by mutations in genes coding for inflammasome components and/or their interaction partners leads to protean inflammatory scenarios associated with fever in children [ 102 , 103 , 104 ]. This family of conditions with seemingly unprovoked attacks of systemic inflammation with neither infectious antigens, nor autoantibodies and autoreactive T cells, is expanding.…”

Section: Conclusive Remarksmentioning

confidence: 99%

The Clinical Chameleon of Autoinflammatory Diseases in Children

Sangiorgi

Rigante

2022

Cells

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The very first line of defense in humans is innate immunity, serving as a critical strongpoint in the regulation of inflammation. Abnormalities of the innate immunity machinery make up a motley group of rare diseases, named ‘autoinflammatory’, which are caused by mutations in genes involved in different immune pathways. Self-limited inflammatory bouts involving skin, serosal membranes, joints, gut and other districts of the human body burst and recur with variable periodicity in most autoinflammatory diseases (ADs), often leading to secondary amyloidosis as a long-term complication. Dysregulated inflammasome activity, overproduction of interleukin (IL)-1 or other IL-1-related cytokines and delayed shutdown of inflammation are pivotal keys in the majority of ADs. The recent progress of cellular biology has clarified many molecular mechanisms behind monogenic ADs, such as familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome (or ‘autosomal dominant familial periodic fever’), cryopyrin-associated periodic syndrome, mevalonate kinase deficiency, hereditary pyogenic diseases, idiopathic granulomatous diseases and defects of the ubiquitin-proteasome pathway. A long-lasting history of recurrent fevers should require the ruling out of chronic infections and malignancies before considering ADs in children. Little is known about the potential origin of polygenic ADs, in which sterile cytokine-mediated inflammation results from the activation of the innate immunity network, without familial recurrency, such as periodic fever/aphthous stomatitis/pharyngitis/cervical adenopathy (PFAPA) syndrome. The puzzle of febrile attacks recurring over time with chameleonic multi-inflammatory symptoms in children demands the inspection of the mixture of clinical data, inflammation parameters in the different disease phases, assessment of therapeutic efficacy of a handful of drugs such as corticosteroids, colchicine or IL-1 antagonists, and genotype analysis to exclude or confirm a monogenic origin.

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Section: Conclusive Remarksmentioning

confidence: 99%

The Clinical Chameleon of Autoinflammatory Diseases in Children

Sangiorgi

Rigante

2022

Cells

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“…Despite evidence to the contrary caregivers still consider childhood fever as a disease (and not as a symptom/sign of an underlying illness); making them to engage in non-evidence based practices ( 9 , 10 ). This wrong perception of fever (that all fevers are harmful) among caregivers has been termed fever phobia or fever anxiety ( 11 ) or fever turmoil ( 2 ). This phobic behavior is pervasive globally among caregivers and healthcare-informed individuals (whether as professionals, parents or undergraduates) ( 12 – 17 ) and transcends race, cultures and whether an individual resides in a resource-rich country or not ( 3 , 6 , 18 , 19 ).…”

Section: Introductionmentioning

confidence: 99%

“…Childhood fever is a naturally conserved immune-protective mechanism aimed at preventing the febrile child from potentially harmful extraneous agents; conferring some survival advantages on the febrile child but occurring at a significant metabolic cost (1,2). It involves a tightly coordinated rise in core body temperature in response to the presence of infectious diseases, inflammatory conditions, malignancy, immunization and following the administration of certain drugs (1,(3)(4)(5) and reportedly accounting for a third of all presenting complaints to pediatricians (6).…”

Section: Introductionmentioning

confidence: 99%

Determinants of caregiver's knowledge and practices regarding childhood fever management in a developing setting: a multi-centre cross-sectional assessment

Ogunyinka,

Oshikoya,

Yusuff

et al. 2023

Front. Pediatr.

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IntroductionFever is both a sign of various diseases (chief of which are infectious in nature) and an adverse effect of certain interventions (e.g. vaccines, drugs) in the pediatric population. It elicits anxiety among caregivers and healthcare professionals alike resulting in non-evidence based practices, adverse medication administration events, waste of scarce resources and overutilization of health facilities. The determinants of these practices among caregivers in the domiciliary contexts have not been well characterized in developing settings.MethodsWe assessed the knowledge and practices of childhood fever and their determinants among caregivers in domiciliary settings in Northern Nigeria using a 41-item questionnaire between August 2020 and February 2021.ResultsThe questionnaire is reliable (knowledge: Cronbach's Alpha = 0.689; practice: Cronbach's Alpha = 0.814) and collected data on a total of 2,400 caregiver-child pairs, who participated in the study. Over two-third (68.3%; 1,640) of the caregivers expressed fever phobic tendencies. Paracetamol was the most commonly used medication and constituted 31.3% of medication administration adverse events reported by the caregivers. Only one out of every six knowledgeable caregivers engaged in evidence-based home childhood fever management practices (7% vs. 41.6%) with being a primary caregiver [Knowledge: odd ratio (OR): 2.81, 95% CI: 0.38; 5.68; p value: 0.04; Practice: OR: 1.65, 95% CI: 0.09; 7.33; 0.02] and having a child/children aged ≤3 years (knowledge: OR: 7.03, 95% CI: 4.89; 9.67, p value: 0.003; practice OR: 3.11, 95% CI: 1.27; 8.59, 0.007) determining both the knowledge and practices of childhood fever management in a household.ConclusionsThe knowledge and practice of childhood fever management among caregivers were sub-optimal with being a primary caregiver and having a child/children aged ≤3 years being the significant determinants of each domain. These gaps underscore the dire need for targeted strategies aimed at improving childhood fever management by educating caregivers.

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“…5 This recurrence of fever flares in children might have dramatic consequences on the overall quality of life of patients and their caregivers, displaying a negative interference with school attendance or daily activities, and might generate a deep sense of frustration in the families. 6 A significantly increased risk of myelodysplastic syndrome and acute myeloid leukemia has been reported for severe congenital neutropenia, but treatment options are limited and no reliable tools predict this kind of progression. Moreover, the delay to a definite diagnosis may be of several months or even years, and children are frequently exposed to redundant and unnecessary diagnostic procedures.…”

Section: Introductionmentioning

confidence: 99%

Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management

Lazzareschi

Rossi

Curatola

et al. 2022

Mediterr J Hematol Infect Dis

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A disparate group of rare hematological diseases characterized by impaired maturation of neutrophil granulocytes defines congenital neutropenias. Neutropenic patients are prone to recurrent infections beginning in the first months of life. Of interest is “cyclic neutropenia”, an ultra-rare disorder revealed by sinusoidal variations of the neutrophil count and periodically-recurring infections every 21 days. Diagnosis of these disorders is frequently obscured by the multiple causes of recurrent fevers in children. Aim of this overview is to outline the physical assessment of children presenting with early-onset symptomatic neutropenia, identify the disease between the many medical conditions and even emergencies which should enter in differential diagnosis, hint at the potential management with granulocyte-colony stimulating factor, define the risk of evolution to hematologic malignancy, and summarize inter-professional team strategies for improving care coordination and outcomes of such patients.

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When, how, and why do fevers hold children hostage?

Cited by 13 publications

References 43 publications

The Clinical Chameleon of Autoinflammatory Diseases in Children

The Clinical Chameleon of Autoinflammatory Diseases in Children

Determinants of caregiver's knowledge and practices regarding childhood fever management in a developing setting: a multi-centre cross-sectional assessment

Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management

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