• Pediatric Infection and Vaccine
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• v.14 no.2
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• pp.194-198
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• 2007

The PFAPA syndrome is characterized by periodic fever, malaise, aphthous stomatitis, pharyngitis and cervical lymphadenitis without long-term sequelae. It has been known as one of the cause of periodic fever in children. Because there is no specific laboratory findings, the diagnosis of PFAPA syndrome is not only made by exclusion of diseases which are related to the other causes of periodic fever, but also made by a careful history and characteristic clinical manifestations. The dramatic response to the single oral dose of corticosteroid is unique to this syndrome. The PFAPA syndrome tends to be resolved without any long-term adverse sequelae. We have reported a 3 year old boy who was diagnosed as having PFAPA syndrome by episodes of periodic fever for 3 months and by excluding other febrile diseases. He was treated with oral prednisolone and his symptoms had been improved dramatically.

• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.991-995
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• 2006

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is one of the causes of periodic fever in pediatrics with unknown etiology. It is characterized by abrupt onset of fever, malaise, aphthous stomatitis, pharyngitis and cervical adenitis without long-term sequelae. Laboratory findings of this sporadic and nonhereditary syndrome are so non-specific that the diagnosis is based on clinical findings. Oral prednisolone is quite effective in controlling the symptoms. We report a case of a 6-year-old girl who was diagnosed as having PFAPA syndrome after 2 years of episodes, by excluding other disease entities with similar clinical features. The patient was treated with oral prednisolone and her symptoms improved dramatically.

• Pediatric Infection and Vaccine
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• v.26 no.3
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• pp.179-187
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• 2019

Purpose: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a leading cause of periodic fever in children. This study describes the clinical characteristics of PFAPA syndrome in patients from a single center. Methods: Thirteen children diagnosed with PFAPA syndrome at Seoul National University Children's Hospital were included in this study. Retrospective medical chart reviews were performed. Results: Among the 13 patients, 8 (61.5%) were male. The median follow-up duration was 3.3 years (range, 10 months-8.3 years). The median age of periodic fever onset was 3 years (range, 1-6 years). All patients had at least 5 episodes of periodic fever and pharyngitis, managed with oral antibiotics, before diagnosis. The median occurrence of fever was every 3.9 weeks and lasted for 4.2 days. All patients had pharyngitis and 12 (92.3%) had cervical lymphadenitis. Blood tests were performed for 12 patients, and no patients had neutropenia. Both the C-reactive protein and erythrocyte sedimentation rate were elevated at medians of 4.5 mg/dL (range, 0.4-13.2 mg/dL) and 29 mm/hr (range, 16-49 mm/hr), respectively. Throat swab cultures and rapid streptococcal antigen tests were negative. Nine (69.2%) patients received oral prednisolone at a median dose of 0.8 mg/kg, and in 6 (66.7%) patients, fever resolved within a few hours. Three (23.1%) patients received tonsillectomy and adenoidectomy. Conclusions: PFAPA syndrome should be considered when a child presents with periodic fever along with aphthous stomatitis, pharyngitis, or cervical lymphadenitis. Glucocorticoid administration is effective for fever resolution and can reduce unnecessary use of antibiotics.

• The Journal of Korean Medicine
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• v.29 no.5
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• pp.134-138
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• 2008

High fever is a common symptom which is considered an important problem, especially in case of undefined causes. Even though most patients generally have been treated by western medicinal treatments, Oriental medicine has developed some prescriptions having therapeutic efficacy of anti-pyrexia. The present study reports a clinical case of prolonged high fever treated by herbal drug, Dalwonum. There was no specific cause shown by blood test, urinalysis or radiologic test to explain the periodic fever over $40^{\circ}C$, but body temperature slowly started normalize after administration of Dalwonum. This study informed us about the potent usefulness of Dalwonum as an anti-pyrexia herbal drug for patients having fever unresponsive to conventional therapies.

• The Journal of Internal Korean Medicine
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• v.19 no.2
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• pp.451-457
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• 1998

This study was performed on the bases of clinical consideration about patient who has malaria. The symptoms of malaria are periodic alternating of chills and fever on everyone, two or three days. The patients feel a chill at the first time of malaria, and have a fever, headache and sweating at the next time. The long cycle of malarial fever such as every 2 or 3 days means that it takes a turn for the worse. In this study, oriental medicine was taken based on the oriental medicine principle such as Dalwonum(達原飮), Sosihotang(小柴胡湯) and Bojungikgitang(補中益氣湯), and the patients take a turn for the better. In the process of treatment if there is no fever in the expected date of the malarial fever, it is sure to have fever in the next expected date. It is favorable that patients lose the chills first and the fever lately in the process of treatment, and we can explain that supporting healthy energy to eliminate evil(扶正祛邪). The long cycle of malarial fever such as every 4, 6 or 9 days means that it is just before the recovery.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.1
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• pp.85-91
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• 2006

PFAPA syndrome is characterized by periodic fevers associated with aphthous stomatitis, pharyngitis, and cervical adenitis and is unusual in infants and children. We report on a case of PFAPA syndrome mimicking cyclic vomiting syndrome in a 42-month-old girl. She had experienced multiple episodes of cyclic vomiting with abdominal pain from age 20 to 30 months. When she was 30 months old, periodic fever with pharyngitis was combined with cyclic vomiting, and when 40 months old, aphthous stomatitis and cervical adenitis were added. These periodic symptoms and signs were not treated with prokinetics or antibiotics. Symptom duration of an episode was 3 days. After cimetidine therapy (150 mg three times daily for 6 months), her febrile and cyclic vomiting episodes ceased. At the time of writing she had not received therapy for 10 months and has remained well without periodic attack.

• Pediatric Infection and Vaccine
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• v.25 no.3
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• pp.113-122
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• 2018

Purpose: The aim of this study was to evaluate the clinical characteristics of children diagnosed as cryopyrin-associated periodic syndrome (CAPS) in Korea. Methods: Diagnosis was made based on clinical features and confirmed by a mutation in the cold-induced autoinflammatory syndrome 1 (CIAS1) gene. Especially, osteocartilaginous overgrowth in the patella or distal femur was so characteristic that its presence warranted a diagnosis of chronic infantile neurologic cutaneous and articular/NOMID. Results: We observed the clinical features of 9 Korean CAPS patients. All the patients suffered from an urticarial rash with recurrent fever. Among the 9 patients, 6 presented with rash and 4 with fever on the 1st or 2nd days of birth. Eight patients showed myalgia, and 7 patients showed arthralgia in the joints, and 6 patients showed radiologic findings of arthropathy including cupping of the metaphysis, excessive growth of the epiphysis, osteopenia or overgrowth of the cartilage. Four patients showed brain atrophy, enlarged ventricles or leptomeningeal enhancement on magnetic resonance imaging. Intellectual disability was observed in 1 patient. Five patients had eye involvement as conjunctivitis, uveitis, chorioretinitis, avascular area or papillary edema, and 3 patients showed progressive hearing loss. All 9 patients showed increased C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Conclusions: All the patients carried a mutation on exon 3 of the CIAS1 gene. After the anakinra (interleukin-1 receptor antagonist) therapy, the fever and rash immediately disappeared, and CRP and ESR were improved.

• Journal of Physiology & Pathology in Korean Medicine
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• v.20 no.1
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• pp.257-261
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• 2006

The symptoms of malaria are periodic alternting of fever and chills on every one, two or three days. The patient has a fever at the first time of malaria, feels a chill, and has muscle pain at the next time. The long cycle of malarial fever such as every 2 or 3 days means that it takes a turn for the worse. In this study, oriental medicine was taken based on the oriental medicine principle such as Gagamcheongbi-tanggami(加減淸脾湯加味方), and the patient takes a turn for the better. The purpose of this paper is to report the patient with malarial fever, a chill and muscle pain who was improved dy oriental medical treatment.

• Journal of Genetic Medicine
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• v.19 no.2
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• pp.57-62
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• 2022

Systemic autoinflammatory diseases (SAIDs) are characterized by unprovoked inflammatory episodes such as recurrent/periodic fever, serositis, skin lesions, abdominal symptoms, arthritis/arthralgia, and central nervous system involvement. Genetic diagnosis of SAIDs has been challenging because disease manifestations overlap among themselves and with other immunological disease categories, such as infection and autoimmune diseases. However, the advent of next-generation sequencing (NGS) technologies and expanding knowledge about the innate immunity and inflammation have made the routine genetic diagnosis of SAIDs possible. Here, we review the recurrent/periodic fevers, other recently identified autoinflammatory diseases, and type I interferonopathies, and discuss the clinical usefulness of NGS targeted sequencing for SAIDs, and recent advance of understandings for this heterogeneous disease group as for underlying primary immunodeficiency.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.4
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• pp.271-277
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• 2018

Purpose: Familial Mediterranean fever (FMF) is an auto inflammatory disease characterized by periodic fever, synovitis and serositis. Patients may be admitted to gastroenterology units due to gastrointestinal symptoms. In this study; we aimed to analyze endoscopic findings and diagnostic utility of endoscopic procedure in children with FMF. Methods: Patient with FMF that was performed endoscopy for the gastrointestinal symptoms were included to the study (39 of 164 patients, 53 procedure). A control group was randomly designed as age and gender matched four endoscopic procedures per one endoscopic procedure of patients with FMF (n=212). Results: No different was found between the patients and control group in esophagogastroscopy findings. However, the diagnosis of gastrointestinal pathology was made by esophagogastroscopy in 46.2% patients. Colonoscopic examination revealed that the frequency of inflammatory bowel disease (IBD) was higher in undiagnosed patients compared to both the control group (50.0% vs. 6.9%, p<0.05, odds ratio [OR]:13.4 and 95% confidence inteval [95% CI]: 2.1-84.3) and the patients under colchicine treatment (50.0% vs. 8.3%, p<0.05, OR: 11 and 95% CI: 0.8-147.8). Colonoscopic procedure that was made after the diagnosis was found to provide contribution by 16.7% in determining the etiology of the additional symptoms. Conclusion: Patients with FMF may be admitted to pediatric gastroenterology outpatient clinic prior to diagnosis or during the follow-up period. The frequency of IBD is high in undiagnosed patients with FMF. Endoscopic procedures may be helpful in these patients for the diagnosis accompanying mucosal lesions.