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Q fever: epidemiology, clinical features and prognosis. A study from 1983 to 1999 in the South of Spain.

OBJECTIVES: Clinical polymorphism is a main feature of Q fever and, depending upon the geographic location, differences in its clinical picture have been described. The objective of this study was to determine the epidemiology, clinical features and prognosis of acute Q fever in our area. METHODS: From 1985 to 1999, consecutive cases of Q fever, presented as febrile syndrome and attended in a tertiary teaching hospital in Sevilla, Spain, were included and followed prospectively. RESULTS: Two hundred and thirty-one cases of acute Q fever were included. A non-focalized febrile syndrome lasting from 7 to 28 days (fever of intermediate duration) was the most frequent presentation (n=208, 90%). One hundred and forty-eight patients had hepatitis. Overall, 53% of the cases were urban and contact with animals was referred in 39% of the patients. No relationship between clinical presentation and possible route of infection was observed. Prognosis was excellent (100% cured), although in 18 patients fever was prolonged more than 28 days and three patients developed life-threatening organ affection. Antimicrobial treatment was more effective if it was administered in the first two weeks (median defervescence of fever: 3 days versus 5.5 days, p<0.01). CONCLUSIONS: Acute Q fever is a common cause of fever of intermediate duration, even in urban areas. Elevation of hepatic enzymes was the most frequent laboratory finding. Severe organ affection is uncommon and the overall prognosis of the disease is excellent. Early treatment seems to shorten the duration of the disease.

Acute Disease↗

Subjective assessment of fever by parents: comparison with measurement by noncontact tympanic thermometer and calibrated rectal glass mercury thermometer.

STUDY OBJECTIVE: To assess the ability of parents to subjectively evaluate their children for fever and to compare their assessments with temperature measurements made with the use of a noncontact tympanic (NCT) or rectal glass mercury thermometer. A secondary goal was to assess how well a recently developed definition of fever for NCT thermometers, when used in the ear-equivalent mode (temperature of 37.7 degrees C or more), performed in a clinical situation. METHODS: This 6-month prospective observational study employed a convenience sample of 180 children, aged birth to 4 years, who presented to the emergency department of a tertiary care children's hospital. Parents were asked to subjectively assess whether their child had a fever. The child's temperature was then measured with an NCT thermometer (three times in the rectal-equivalent mode and three times in the actual-ear mode). Both the subjective assessment and the NCT temperatures were compared with the rectal temperature measured by a rectal glass mercury thermometer. RESULTS: The mean age of participants was 14.6 +/- 11.8 months (range, 2 days to 48 months); 56% were boys. The sensitivity of parental detection of fever by subjective means was 81.8% and the specificity 76.5%. The parent and the rectal glass thermometer agreed 79% of the time (95% confidence interval [CI], 73% to 85%). The sensitivity of the first temperature reading obtained with the NCT thermometer in rectal-equivalent mode was 74.7%, and the specificity was 96.3%. The NCT thermometer and the rectal glass thermometer agreed 84% of the time (95% CI, 78% to 89%). Use of the proposed definition of fever for NCT thermometers, when used in the ear-equivalent mode, caused sensitivity of a single measurement for fever to drop to 53.5%. CONCLUSION: Parental subjective assessment of fever agreed with the presence of fever as measured by rectal glass thermometer in 79% of cases. Specificity was improved with the use of the NCT thermometer. The recently proposed definition for fever for NCT thermometers, when they are used in the ear-equivalent mode, does not appear to be validated by the current data.

Child, Preschool↗

Are circulating cytokines interleukin-6 and tumor necrosis factor alpha involved in chlorpyrifos-induced fever?

Oral exposure to chlorpyrifos (CHP) in the rat results in an initial hypothermic response followed by a delayed fever. Fever from infection is mediated by the release of cytokines, including interleukin-6 (IL-6) and tumor necrosis factor (TNF alpha). This study determined if the CHP-induced fever involves cytokine-mediated mechanisms similar to that of infectious fevers. Long-Evans rats were gavaged with the corn oil vehicle or CHP (10-50 mg/kg). The rats were euthanized and blood collected at various times that corresponded with the hypothermic and febrile effects of CHP. Plasma IL-6, TNF alpha, cholinesterase activity (ChE), total iron, unsaturated iron binding capacity (UIBC), and zinc were measured. ChE activity was reduced by approximately 50% 4 h after CHP. There was no effect of CHP on IL-6 when measured during the period of CHP-induced hypothermia or fever. TNF alpha levels nearly doubled in female rats 48 h after 25 mg/kg CHP. The changes in plasma cytokine levels following CHP were relatively small when compared to > 1000-fold increase in IL-6 and > 10-fold rise in TNF alpha following lipopolysaccharide (E. coli; 50 microg/kg; i.p.)-induced fever. This does not preclude a role of cytokines in CHP-induced fever. Nonetheless, the data suggest that the delayed fever from CHP is unique, involving mechanisms other than TNF alpha and IL-6 release into the circulation characteristic of infectious fevers.

Animals↗

Nursing practice for HIV/AIDS fever care: a descriptive study.

Nurses often perceive fever in relation to the impact it has on specific patient populations. Fever in HIV/AIDS patients is a common symptom. This descriptive study explored how nurses in AIDS care defined and described fever in HIV/AIDS patients and used nursing interventions for fever management. Seventy-five registered nurses, nurse practitioners, and LPNs employed in AIDS care throughout Florida were surveyed concerning interventions for AIDS-related fever. The study revealed that nurses in AIDS care treat fever by providing patient comfort, initiating nursing interventions for low-grade to moderate fever, and notifying the primary care provider if nursing interventions do not keep the temperature below 100.9 degrees F. The sample provided a variety of nursing interventions to make the patient more comfortable. In addition, the study also revealed a unique perspective for fever care, which influences the practice of nurses in AIDS care and might differentiate nursing practice in AIDS care from other nursing specialties. However, some interventions differed from fever nursing management strategies published by the Association of Nurses in AIDS Care. Further research needs to explore the efficacy of these interventions.

Acquired Immunodeficiency Syndrome↗

A peripheral mechanism of fever: differential sensitivity to the antipyretic action of methyl scopolamine.

The organophosphate pesticide (OP) chlorpyrifos leads to an acute period of hypothermia followed by a delayed fever in the rat. Methyl scopolamine, a peripheral muscarinic antagonist, is thought to have little effect on body temperature of the rat because it does not cross the blood brain barrier. However, administration of methyl scopolamine (1 mg/kg, i.p.) during the period of chlorpyrifos-induced fever results in a rapid recovery of core temperature. This indicates a peripheral cholinergic pathway is operative in the febrile response to chlorpyrifos and possibly other modes of fever. In this study, we evaluated the possible antipyretic role of methyl scopolamine (i.p.) to a variety of stimuli that lead to fever-like responses in the rat: stress-induced (handling and cage switch), chlorpyrifos-induced (15 mg/kg, p.o.), nocturnal-induced, and lipopolysaccharide (LPS)-induced fever (50 microg/kg, i.p.). Methyl scopolamine led to marked reversal in the elevated core temperature caused by handling, cage switch, and during the nocturnal phase. It is of interest to note that all these elevations of core body temperature are prostaglandin mediated and are blocked with the antipyretic drug, sodium salicylate. However, LPS-induced fever, also a prostaglandin dependent fever, was unaffected by methyl scopolamine. Methyl scopolamine also lowered baseline core temperature when administered during the afternoon, but not during the morning in unstressed animals. It is proposed that a peripheral cholinergic pathway, possibly mediated through afferent vagal pathways, is operative in controlling core temperature during fevers associated with stress, nocturnal phase, and a pesticide. During recovery from exposure to a LPS, the fever appears to be mediated independently of peripheral cholinergic activation.

Animals↗

[Fever--useful or noxious symptom that should be treated?].

Fever is a phylogenetically ancient host reaction to invading microorganisms and other noxious stimuli. Poikylothermic organisms can reach febrile temperatures by seeking a hot environment in response to a higher set point in their thermoregulatory center. Endothermic organisms produce febrile temperatures through endogenous heat production at the expenditure of a higher metabolic rate. Nevertheless, fever has been conserved during evolution through millennia, obviously because of its advantage for host defense. Despite of these arguments most doctors, nurses and patients treat fever with antipyretics. The role of fever for the recovery from low risk infections is marginal at best. A large study of ibuprofen in patients with severe sepsis could not establish a positive or negative role on the course or final outcome of the infection in an intensive care setting. These clinical observations seemingly contradict findings in severe experimental bacterial infections in rodents but it has to be taken into consideration that these animals, in contrast to patients, received no antibiotic treatment. In patients with influenza-like illnesses non-steroidal antirhumatics (NSAR) improve fever and wellbeing with little or no evidence for undesired side-effects. It therefore appears appropriate to treat patients with these and similar infections with NSAR. Antipyretic therapy in special patient groups such as brain injury victims, patients with cardiac or respiratory failure or dementia has not been established to be indicated to overcome a worsening of these organs to fail during infections. In children with a history of fever convulsions prevention or lowering of fever does not reduce recurrence. In patients with strokes it appears advisable however to use antipyretics in case of fever despite of a present lack of a proven beneficial effect. In conclusion symptomatic antipyretic therapy should be considered for low risk infections if patient suffering from fever. For more severe infections antipyretic therapy can be applied on an individual basis without too much hope to improve outcome or cause a severe worsening of prognosis.

Analgesics, Non-Narcotic↗

Accuracy of perception and touch for detecting fever in adults: a hospital-based study from a rural, tertiary hospital in Central India.

OBJECTIVES: To evaluate the diagnostic accuracy of the patient's perception, and of the touch of patient attendants and a doctor for detecting fever. METHODS: We enrolled patients older than 13 years who presented with history of fever to the in- and out-patient departments of a rural teaching hospital. The design was a double-blind, cross-sectional analysis of a hospital-based case series, independently comparing reported history of fever and touch of patient attendant and that of doctor against an established reference standard (axillary temperature > 37.5 degrees C). Diagnostic accuracy was measured by computing sensitivity, specificity, and likelihood ratio values. The agreement between the patient, his attendant and the doctor was assessed by kappa statistic. RESULTS: We studied 462 patients of whom 274 (59.3%) were men. A total of 206 patients (44.58%) had fever. The patient's perception of fever (LR+ 1.77; 95% CI 1.52, 2.06), patient attendant's touch (LR+ 2.03; 95% CI 1.74, 2.36) and the doctor's touch (LR+ 3.08; 95% CI 2.51, 3.71) did not accurately distinguish those with and without fever. Doctors (LR- 0.20; 95% CI 0.17, 0.34) and patient attendants (LR- 0.24; 95% CI 0.14, 0.28) were more accurate in ruling out fever. The patient's perception agreed moderately with patient attendant's touch (kappa = 0.44; 95% CI 0.36, 0.53), and the doctor's assessment (kappa = 0.47; 95% CI 0.39, 0.55). There was moderate agreement between patients' attendants and the study doctor (kappa = 0.48; 95% CI 0.40, 0.56). CONCLUSIONS: Our findings suggest that patients, their attendants or doctors cannot accurately detect the presence of a fever without using a thermometer. Doctors should confirm a history of fever by recording temperature.

Adolescent↗

Evaluation of postoperative fever: usefulness and cost-effectiveness of routine workup.

OBJECTIVE: This study was undertaken to determine which investigative tools are of most clinical benefit in a routine postoperative fever workup and to describe which postoperative patients require closer investigation at the time of fever. STUDY DESIGN: We performed a retrospective analysis of 676 patients undergoing exploratory laparotomy for a gynecologic condition who had a fever of 38.0 degrees C (100.4 degrees F) or higher. Charts were reviewed for independent variables related to their diagnosis and surgery in addition to any workup for fever. An estimate of the total charge of a routine workup was performed. Statistical analysis was performed with SPSS for Windows, version 10.1. RESULTS: We found 194 patients (29%) had a postoperative temperature of 38.0 degrees C or higher. A laboratory/radiographic workup for the fever was performed in 119 women (61%). Only 15% of urinalyses, 14% of urine cultures, 7% of blood cultures, 14% of chest radiographs, and 17% of sputum cultures were positive. There were several significant differences between those with and without serious infections that can guide management of postoperative patients. The total charge for laboratory/radiographic workup was 48,432 US dollars or 2201 US dollars per serious infection diagnosed. CONCLUSION: Approximately 29% of women undergoing laparotomy by gynecologists have a fever of 38.0 degrees C (100.4 degrees F) or higher. We found the majority of fever workups were of low clinical yield and added moderate cost. Significant infection is associated with surgery for malignancy, bowel resection, number of febrile days, higher fever, and moderately increased white blood cell count. Only patients with these characteristics are most likely to benefit from laboratory and/or radiographic workup of fever.

Adolescent↗

Risks factors and prevention of Q fever endocarditis.

Coxiella burnetii causes acute and chronic Q fever. To evaluate the risk factors of development of chronic endocarditis following Q fever and to assess the best preventive therapy, a retrospective study of patients diagnosed as having Q fever during 1985-2000 was conducted. Twelve patients with acute Q fever who developed endocarditis and 102 patients with Q fever endocarditis were included in the study. When compared to 200 control patients with acute Q fever, preexisting valvular disease (P<10(-7)), especially a prosthetic valve (P=.01), were encountered more often among patients with endocarditis. Among patients with valvular defects, we estimate the risk of developing endocarditis to be 39%. A combination of doxycycline plus hydroxychloroquine was better at preventing the development of endocarditis than doxycycline alone (P=.009). Our results should encourage physicians to detect valvular lesions in patients with acute Q fever and to search for acute Q fever in patients with a valvulopathy and unexplained fever. A proper treatment for such patients and a scheduled follow-up should reduce the risk of developing endocarditis.

Aged↗

Lassa fever in Guinea: I. Epidemiology of human disease and clinical observations.

The arenavirus Lassa is found in West Africa, where it sometimes causes a severe illness called Lassa fever. Lassa fever has been seldom investigated outside of a few hyperendemic regions, where the described epidemiology may differ from that in areas of low or moderate incidence of disease. Through a prospective cohort study, we investigated the epidemiology and clinical presentation of Lassa fever in Guinea, where the disease has been infrequently recognized. A surveillance system was established, and suspected cases were enrolled at five Guinean hospitals. Clinical observations were made, and blood was taken for enzyme-linked immunosorbent assay testing and isolation of Lassa virus. Lassa fever was confirmed in 22 (7%) of 311 suspected cases. Another 43 (14%) had Lassa IgG antibodies, indicating past exposure. Both sexes and a wide variety of age and ethnic groups were affected. The disease was more frequently found, and the IgG seroprevalence generally higher, in the southeastern forest region. In some areas, there were significant discrepancies between the incidence of Lassa fever and the prevalence of antibody. Clinical presentations between those with Lassa fever and other febrile illnesses were essentially indistinguishable. Clinical predictors of a poor outcome were noted, but again were not specific for Lassa fever. Case-fatality rates for those with Lassa fever and non-Lassa febrile illnesses were 18% and 15%, respectively. Seasonal fluctuation in the incidence of Lassa fever was noted, but occurred similarly with non-Lassa febrile illnesses. Our results, perhaps typical of the scenario throughout much of West Africa, indicate Lassa virus infection to be widespread in certain areas of Guinea, but difficult to distinguish clinically.

Adolescent↗

Persistent fever in association with infective endocarditis.

Fever persisting despite adequate antimicrobial therapy for endocarditis can be an ominous sign. To evaluate the significance of persistent fever in this situation, we reviewed the records of patients at three hospital affiliates of Albert Einstein College of Medicine. Twenty-six patients with 27 episodes of endocarditis and fever lasting for > or = 2 weeks despite appropriate antimicrobial therapy were identified and compared with a matched cohort of 26 patients with endocarditis but without prolonged fever. The median duration of fever in the former group was 35 days. Cardiac infection caused fever in 13 of these patients, seven of whom had myocardial abscesses. Additional causes of infection included drug treatment, nosocomial transmission of pathogens, and pulmonary emboli. Sixteen patients required cardiac surgery (seven on an emergent basis), whereas only two controls underwent such a procedure (P < .001). Twenty-two patients with persistent fever and five controls developed nosocomial complications (P < .001). Six patients with fever died, five from endocarditis-related complications. Thus persistent fever often indicates complicated endocarditis. We present an approach for the evaluation of the patients affected by this condition.

Adolescent↗

The cause of fever following resolution of neutropenia in patients with acute leukemia.

Many studies have examined the etiology of fever complicating neutropenia. Little is known about the etiology of fever occurring immediately following recovery from myelosuppression. We reviewed 165 episodes of fever in patients who were admitted to the University of Pennsylvania Medical Center (Philadelphia) between 1 August 1992 and 15 August 1994 for the treatment of acute leukemia. We included patients who had episodes of fever (temperature of > or = 38 degrees C) for > or = 48 hours within 10 days after an absolute neutrophil count of < or = 500 cells/mm3 was determined. Twenty-nine (20%) of 145 episodes met these criteria. In 5 (17%) of 29 episodes the cause of fever was a bacterial infection, in 6 (21%) of 29 episodes the cause of fever was noninfectious, and in 12 (41%) of 29 episodes the cause of fever was unknown. Six (21%) of 29 episodes were due to documented or suspected fungal infection, four were due to suspected pulmonary aspergillosis, and two were due to systemic candidal infections. Fever following recovery from chemotherapy-induced neutropenia is common. Fungal infections occur frequently after recovery from myelosuppression despite widespread use of empirical and prophylactic antifungal therapy. Improved strategies for diagnosing and preventing fungal infections in patients who have fever following recovery from myelosuppression are clearly needed.

Adolescent↗

Seasonality and malaria in a west African village: does high parasite density predict fever incidence?

In this cohort study, the authors studied the effect of blood malaria parasite density on fever incidence in children in an endemic area with 9 days' follow-up of 1- to 12-year-old children during two time periods: the end of the dry season (May 1993: n = 783) and the end of the rainy season (October 1993: n = 841) in Bougoula, West Africa (region of Sikasso, Mali). The cumulative incidence of fever (temperature > 38.0 degrees C) was 2.0% in the dry season and 8.2% in the rainy season (p < 0.0001). In the rainy season, the risk of fever was increased in children of ages 1-3 years (relative risk (RR) = 2.5, 95% confidence interval (CI) 1.6-4.1); in those with an initial parasitemia > 15,000/microliter (RR = 2.7, 95% CI 1.4-5.4); in children with an enlarged spleen (RR = 2.0, 95% CI 1.2-3.3); or in those with anemia (hematocrit < 30%: RR = 1.8, 95% CI 1.1-2.9). In the dry season, anemia was the only predictor of fever incidence. In the rainy season, the best predictors of fever were, in order, age (< 4 years), enlarged spleen, and high parasite density. Even in the higher risk groups, the cumulative incidence was < 20%. The authors conclude that most children with high parasite density do not develop fever subsequently. The association between parasite density and fever varies according to age and season. Since even high levels of parasite density do not reliably predict fever incidence, parasite density should be considered as just one of a group of indicators that increase the probability of a fever of malarial origin.

Africa, Western↗

Fever during pregnancy and spontaneous abortion.

The hypothesis that maternal fever during pregnancy is a risk factor for spontaneous abortion was tested in a case-control study by comparing the frequencies and timing of fevers of 100 F (37.78 C) or more among three groups of women: women having euploid abortions, women having aneuploid abortions, and women delivering at 28 weeks gestation or later (controls). Cases and controls were identified in three New York City hospitals between August 1979 and June 1982. It was hypothesized that if fever was an antecedent, rather than a symptom of spontaneous abortion, an association would be detected with euploid but not with aneuploid abortions. Among public patients, reported fevers were significantly more frequent among euploid abortions than among controls (18% vs. 7.1%, odds ratio = 2.96), whereas reported fevers were not more frequent among aneuploid abortions (3.9% vs. 7.1%, odds ratio = 0.52). It was also postulated that variation in the strength of the association of fever with euploid abortion with the duration of the interval between the fever and the abortion might yield insight about mechanisms underlying an association. The odds ratios for fever occurring at three intervals--in the same calendar month as a euploid abortion, one month before, and two or more months before--were 6.04, 3.28, and 1.41, respectively. The findings for private patients--for whom a control group was not recruited--are compatible with those for public patients although not statistically significant: the odds of fever with euploid abortion were 2.18 times those with aneuploid abortion.

Abortion, Spontaneous↗

Mothers' perceptions of fever in children.

The fever is one of the commonest symptoms of disease in childhood. A mother's knowledge and conception of fever may determine the degree of her anxiety and fear, and reflect on the way the fever is managed at home. Our understanding of mothers' attitudes may help to improve the home management of fever by correcting unrealistic beliefs. Mothers of 100 children were randomly selected and interviewed to answer a questionnaire about fever. Sixty mothers thought that fever could lead to death, others thought that it might lead to paralysis, mental retardation or brain damage. This may explain their undue anxiety and fear when having a child with fever. Cold exposure, teething, exposure to sunlight and having a warm drink were considered as causes. A minority reduced the child's clothes during fever. Tap water sprinkling and occasionally alcohol were used. Ninety-four mothers used antipyretics, all used paracetamol and 23 per cent used aspirin. Sixty two used antibiotics indiscriminately. Improved health education is required to allay mothers fear and anxiety and promote a more appropriate fever management at home.

Analgesics, Non-Narcotic↗

Cytarabine-induced fever complicating the clinical course of leukemia.

The aim of this study is to assess the frequency and clinical characteristics of cytosine arabinoside-induced fever in patients with acute myeloid leukemia in remission, receiving high-dose (3 g/m2) consolidation therapy. We have investigated 77 consolidation cycles over a study period of 4 years. A strict definition of cytosine arabinoside-induced fever (i.e. patients without neutropenia and with negative blood cultures during the fever episodes) was used. Of the 77 consolidation cycles, fever due to cytarabine was detected in 33 cycles (43%). Median time of onset of fever from the beginning of first chemotherapy dose was 22 h and maximum temperature was in the range 38.0-39.7 degrees C (mean+/-SD: 38.8+/-0.5 degrees C). Median duration of fever was 10.15 h and did not exceed 72 h. There was no difference with regard to neutrophil and white blood cell counts between cycles with or without cytarabine fever. The cost of investigation of fever source was about US$2137. Our analysis suggests that 'cytarabine fever' is a frequent and often a self-limiting complication of high-dose cytosine arabinoside consolidation therapy, and cost-reductive approaches could be structured based on this background.

Acute Disease↗

Pediatric emergency department nurses' perspectives on fever in children.

BACKGROUND: Fever is the most common complaint of children seen in a Pediatric Emergency Department (PED). Since pediatric emergency nurses commonly educate parents on fever management, this study sought to examine their knowledge base regarding fever in children. METHODS: Through convenience sampling, pediatric emergency registered nurses working at one of four PEDs were surveyed using a self-administered questionnaire containing 10 open-ended questions pertaining to fever in children. RESULTS: Eighty-eight pediatric emergency registered nurses (median experience 8.0 years, range 3 months to 28 years) were surveyed. The median temperature considered by pediatric emergency nurses to be a fever was 38.0 degrees C (100.4 degrees F) with a range of 37.2 degrees C (99.0 degrees F) to 38.9 degrees C (102.0 degrees F), while the median temperature considered to be dangerous to a child was 40.6 degrees C (105.0 degrees F) with a range of 38.0 degrees C (100.4 degrees F) to 41.8 degrees C (107.0 degrees F). Eleven percent was not sure what temperature constituted a fever while 31% was not sure what temperature would be dangerous to a child. Fifty-seven percent considered seizures the primary danger to a febrile child while 29% stated permanent brain injury or death could occur from a high fever. Sixty percent chose acetaminophen as first line treatment while 7% stated alcohol or tepid water baths were also acceptable treatment options. Thirty-eight percent stated that a different medication should be added if a child was still febrile 1 hour after initial treatment while 31% would not use additional medication. Eighteen percent stated it was dangerous for a child to leave the PED if still febrile. CONCLUSION: Fever phobia and inconsistent treatment approaches occur among experienced pediatric emergency registered nurses. These phobias and inconsistencies subsequently could be conveyed to parents. In order to assure accurate parental education, PEDs should educate their medical team regarding the management of fever in children.

Analgesics, Non-Narcotic↗

Periodic fever, aphthous stomatitis, pharyngitis, adenitis: a clinical review of a new syndrome.

Periodic fevers (fevers that occur predictably at fixed intervals) are unusual in infants and children. The classic periodic fever syndrome is cyclic neutropenia (neutropenia followed by infections and fever that recur every 21 days). A new periodic fever syndrome PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) has been characterized over the past decade. PFAPA is defined clinically, because specific laboratory abnormalities have not been found. The clinical characteristic of PFAPA is high fevers (usually 40.0 degrees C to 40.6 degrees C) recurring at fixed intervals every 2 to 8 weeks. The fevers last for about 4 days, then resolve spontaneously. Associated with the fevers are aphthous stomatitis in 70% of patients, pharyngitis in 72% of patients, and cervical adenitis in 88% of patients. PFAPA is not familial and begins before the age of 5 years. An episode of PFAPA can be aborted with one or two small doses of prednisone. The episodes of PFAPA may last for years and the patient is well between episodes. The cause of PFAPA is unknown and there are no reported sequelae.

Child↗