A clinical review of 105 patients with PFAPA (a periodic fever syndrome).

AIMS: We describe the presentations and clinical outcomes of pediatric patients diagnosed with PFAPA (Periodic Fever, Aphthous lesions, Pharyngitis, and cervical Adenitis). MATERIALS AND METHODS: The medical records of children with recurrent fever and referred between 1998 and 2007 to a tertiary pediatric care hospital were reviewed. Children who met clinical criteria for PFAPA were then asked to participate in a follow-up study. RESULTS: One hundred and five children met study criteria for PFAPA which included at least six episodes of periodic fever. Most (62%) were males, the mean age at onset of PFAPA was 39.6 months (80% were <5 years at onset), the mean duration of individual fever episodes was 4.1 days, and the mean interval between episodes was 29.8 days. Accompanying signs and symptoms included aphthous stomatitis (38%), pharyngitis (85%), cervical adenitis (62%), headache (44%), vomiting with fever spikes (27%) and mild abdominal pain (41%). A prodrome (usually fatigue) preceded the fever in 62% of patients. Parents noted that when their child with PFAPA had fever, other family members remained well. Laboratory tests in patients with PFAPA were nonspecific. Individual episodes of fever usually resolved with a single oral dose ( approximately 1 mg/kg) of prednisilone. The interval between fever episodes shortened in 50% of patients who used prednisilone. PFAPA resolved spontaneously (mean length 33.2 months) in 211105 (20%) patients. PFAF'A episodes continued (mean length 23 months) at the end of this study in 661105 (63%) patients. Cimetidine therapy was associated with the resolution of the fevers in 7/26 (27%) patients; tonsillectomy was associated with the resolution of the fevers in 11/11 (100%) patients. CONCLUSION: PFAPA can usually be defined by its clinical characteristics. Individual febrile episodes usually resolve dramatically with oral prednisilone. The cause of PFAPA is unknown and research is needed to define its etiology. The overall prognosis for children with PFAPA is excellent.

Management of tick bites and early Lyme disease: a survey of Connecticut physicians.

Practice guidelines have been published for the treatment of Lyme disease (LD). These guidelines have been challenged as inadequate. Two common LD management problems are antibiotic prophylaxis of deer tick bites (deer ticks may carry Borrelia burgdorferi, the spirochete that causes LD) and antibiotic treatment of erythema migrans, the pathognomonic rash of LD. A 1-page questionnaire was sent to a 13% (573/4300) sample of Connecticut physicians to define how they treat deer tick bites and erythema migrans. Questionnaires were returned by 320 (56%) of 573 physicians. Questionnaires were analyzed for the 267 physicians who saw patients with LD. Seventy (26%) of the 267 surveyed physicians prescribed antibiotic prophylaxis for patients with tick bites. B burgdorferi serology was ordered by 31% of physicians for patients with tick bites. Most surveyed physicians treated erythema migrans with doxycycline or amoxicillin for a mean of 21 days. Serology was ordered by 49% of physicians for patients with erythema migrans. Most physicians did not use prophylaxis for patients with deer tick bites. In addition, most of the physicians surveyed followed established guidelines for treating patients with erythema migrans. However, many of the physicians surveyed do serologic testing for patients with tick bites and/or erythema migrans. Serologic testing for these patients is usually not necessary.

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.

Methicillin-resistant Staphylococcus aureus infections in 2 pediatric outpatients.

Methicillin-resistant Staphylococcus aureus (MRSA) infections are an emerging problem in children. The following are 2 case reports of unsuspected MRSA infections: the first is an infant with cervical adenitis and the second is a child with a deep infection of the toe. Both patients failed outpatient therapy with oral cephalosporins and required hospitalization for surgical drainage. Both patients had cultures positive for MRSA at surgery. Neither patient had any risk factors for acquiring MRSA. Thus, outpatients with presumed staphylococcal infections who fail oral therapy with cephalosporins may be infected with MRSA.

Immunogenicity of a recombinant Borrelia burgdorferi outer surface protein A vaccine against Lyme disease in children.

BACKGROUND AND OBJECTIVE: A recombinant lipoprotein vaccine against Lyme disease, containing 30 microg of Borrelia burgdorferi outer surface protein A (OspA) with aluminum adjuvant, has been shown in a large US field trial of subjects >/=15 years of age to offer 76% efficacy against clinical Lyme disease after 3 injections given at 0, 1, and 12 months. Lyme disease is also an important problem in children; thus, OspA vaccine trials in children are needed. The purpose of this study was to investigate the safety and immunogenicity of 2 different doses of lipoprotein OspA with aluminum adjuvant vaccine in healthy children 5 to 15 years of age in a double-blind, randomized study. STUDY DESIGN: In a double-blind study, 250 children from the Czech Republic were randomly assigned to receive 15 microg or 30 microg of OspA vaccine at 0, 1, and 2 months. Serum samples, obtained before vaccination and 1 month after the second and third doses, were analyzed for antiOspA antibody. Solicited and unsolicited symptoms were collected from diary cards. RESULTS: Local pain at the injection site was reported by approximately 76% of the 250 children. Headaches (after 5% to 18% of the injections) and malaise (after 2% to 16% of the injections) were the most frequently reported general symptoms. Local and generalized symptoms were not different between the 15 microg and 30 microg groups, and all symptoms resolved within 4 days. Both doses were highly immunogenic, with the 30 microg dose eliciting higher antibody levels. Seroconversion occurred in 99% of the 250 children. CONCLUSIONS: The OspA vaccine against Lyme disease was well tolerated and highly immunogenic in children.

Periodic fever syndrome in children.

OBJECTIVES: To describe the presentation, clinical course, therapeutic response, and long-term follow-up of patients with a syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA). STUDY DESIGN: Patients with PFAPA (n = 94) referred over a 10-year period completed a registry form and provided medical records. Follow-up telephone calls were made in late 1997 to determine the persistence of episodes and sequelae. RESULTS: PFAPA episodes lasted 4.8 days (95% confidence interval 4.5 to 5.1) and recurred every 28 days (confidence interval 26 to 30), with a maximal temperature of 40.5 degrees C (confidence interval 40. 4 degrees to 40.6 degrees ). Of the 83 children available for follow-up, 34 no longer had episodes. In the remainder the episodes did not differ in character but recurred less frequently over time. The affected children had no long-term sequelae. Glucocorticoids were highly effective in controlling symptoms. Tonsillectomy and cimetidine treatment were associated with remission in a small number of patients. CONCLUSIONS: PFAPA is a not uncommon cause of periodic fever in children. In some children the syndrome resolves, whereas symptoms in others persist. Long-term sequelae do not develop. The syndrome is easily diagnosed when regularly recurring episodes of fever are associated with aphthous stomatitis, pharyngitis, or cervical adenitis.

Once-daily therapy for streptococcal pharyngitis with amoxicillin.

OBJECTIVE: An orally administered antimicrobial regimen for the treatment of group A beta-hemolytic streptococcal (GABHS) pharyngitis given once rather than multiple times each day would be more convenient and might result in improved patient compliance. The purpose of this study was to evaluate the effectiveness of once-daily amoxicillin in the treatment of GABHS pharyngitis. PATIENTS: Children presenting to a private pediatric office with GABHS pharyngitis. DESIGN: Patients were randomly assigned to receive orally either amoxicillin (750 mg once daily) or penicillin V (250 mg three times a day) for 10 days. Compliance was monitored by urine antimicrobial activity. OUTCOMES: Outcomes were measured by impact on the clinical course, eradication of GABHS within 18 to 24 hours, and bacteriologic treatment failure rate as determined by follow-up throat cultures 4 to 6 and 14 to 21 days after completing therapy. GABHS isolates were serotyped to distinguish bacteriologic treatment failures (same serotype as initial throat culture) from new acquisitions (different serotypes). RESULTS: During the 16 months of this study, 152 children between 4 and 18 years of age (mean, 9.9 years) were enrolled; 79 children were randomly assigned to receive once-daily amoxicillin and 73 were assigned to receive penicillin V three times a day. The children in the two treatment groups were comparable with respect to age, duration of illness before initiation of therapy, compliance, and signs and symptoms at presentation. There was no significant difference in the clinical or bacteriologic responses of the patients in the two treatment groups at the 18- to 24-hour follow-up visit. Bacteriologic treatment failures occurred in 4 (5%) of the 79 patients in the amoxicillin group and in 8 (11%) of the 73 patients in the penicillin V group. CONCLUSIONS: These data demonstrate that once-daily amoxicillin therapy is as effective as penicillin V therapy given three times a day for the treatment of GABHS pharyngitis, and if confirmed by additional investigations, once-daily amoxicillin therapy could become an alternative regimen for the treatment of this disease.

Rabies prophylaxis following the feeding of a rabid pony.

A survey was performed to identify people who were exposed to a rabid pony and determine whether or not they received rabies postexposure prophylaxis (PEP). Sixty-one visitors who came in contact with the rabid pony were identified. These visitors heard about the rabid pony via the news media. Forty-five visitors were exposed during the 2 weeks before the pony died. Thirty-two of these 45 visitors received PEP. Thirty-one visitors had or may have had saliva contact to an open wound or mucosa and all 31 received PEP. Fourteen visitors had no saliva contact to a wound or mucosa and one received PEP. Sixteen visitors were exposed before the pony was potentially shedding rabies virus and one received PEP. No visitor was bitten by the pony. Most of the persons 31/33 (94%) who received PEP had an exposure for which PEP was indicated. Nonbite transmission of rabies is discussed.

Addition of rifampin to cephalexin therapy for recalcitrant staphylococcal skin infections--an observation.

We report two pediatric patients with recalcitrant staphylococcal infections whose infections resolved when rifampin was added to standard antistaphylococcal therapy. One patient had a post-varicella staphylococcal ulcerative lesion and did not respond to cephalexin alone but did respond when rifampin was added. A second patient had staphylococcal bullous impetigo and did not respond to dicloxacillin or cephalexin but did respond when rifampin was added to the cephalexin. If a patient fails to respond to traditional antistaphylococcal therapy, the addition of rifampin may be beneficial.