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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.116, No.5, May 2012
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Original Article
Title
Clinical Features of 20 Patients with Periodic Fever with Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome
Author
Aika Watanabe Masao Adachi Mitsuro Kobayashi Yoshinobu Oyazato Atsushi Nishiyama Ichiro Kamioka and Masahiko Yonetani
Department of Pediatrics, Kakogawa City Hospital Organization, Kakogawa West-City Hospital
Abstract
PFAPA syndrome has been described as a non-infectious, non-autoimmune, and autoinflammatory disease, taking the form of periodic fever associated with aphthous stomatitis, pharyngitis, and cervical adenitis. We treated 20 cases of PFAPA syndrome in the past two years. All of the cases met the clinical criteria purposed by Thomas (1999), and included 8 boys, 10 girls and 2 women. Mean onset age was 1.8 years, and the median interval between febrile attacks was 35 days. Clinical symptoms during febrile periods consisted of pharyngitis (100%), aphthous stomatitis (85%), cervical adenitis (80%), and the presence of preliminary symptoms preceding each fever (45%). The existence of these preliminary symptoms was effective in differentiating febrile attacks caused by PFAPA syndrome from those caused by other agents (i.e., infection).
Oral prednisolone (PSL) treatment was initiated in 10 cases and obtained dramatic responses, resulting in improved quality of life both in these patients and their families, and cutting down medical expenses as well. In eight of these cases undergoing PSL therapy, however, the intervals between febrile attacks shortened, causing us to recognize the limitations of long-term use of PSL in the treatment of PFAPA syndrome. In addition, treatment with cimetidine in 3 cases and with famotidine in 2 cases produced no effect, but tonsillectomy in 2 cases succeeded in decreasing the incidence of febrile episodes.
In the present investigation, we identified several unique features in PFAPA syndrome, including gender differences, some familial cases, individual preliminary symptoms, clinical profiles in cases with hyperimmunoglobulinemia D, and the variability of PSL therapy. Many more cases like those in the present study should be reported to understand the pathophysiology and the genetic background of this little-known syndrome.
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Original Article
Title
Clinical Experience of Sodium Phenylbutyrate in Patients with Ornithine Transcarbamylase Deficiency in Japan
Author
Shoko Komatsuzaki1) Toshihiro Ohura2)3) Osamu Sakamoto2) Masaki Takayanagi4) Toju Tanaka5) Torayuki Okuyama6) Fumio Endo7) and Yoichi Matsubara1)
1)Department of Medical Genetics, Tohoku University School of Medicine
2)Department of Pediatrics, Tohoku University School of Medicine
3)Division of Pediatrics, Sendai City Hospital
4)Division of Metabolism, Chiba Children's Hospital
5)Departments of Medical Genetics, National Center for Child Health and Development
6)Clinical Laboratory Medicine, National Center for Child Health and Development
7)Departments of Pediatrics, Kumamoto University School of Medicine
Abstract
Background: Urea cycle disorders (UCDs) can be treated by a low protein diet, arginine or citrulline supplement, and drugs for alternative pathway therapy (sodium benzoate and sodium phenylbutyrate (NaPB)). NaPB is approved as a treatment for UCDs in the United States, Europe, and Korea, but not in Japan.
Object: Our group evaluated the therapeutic and adverse effects of NaPB in Japanese patients. We performed a prospective study in 6 patients with ornithine transcarbamylase deficiency (2 males and 4 females). All of the patients were treated by a low protein diet, sodium benzoate, and either arginine or citrulline supplement up to the start of the study. The treatment period was commenced by switching the patients from sodium benzoate to NaPB.
Results: Plasma ammonia levels, amino acids, and nutritional status were evaluated after 12 months of treatment with NaPB. Difficulty in controlling hyperammonemia (150-330 μg/dl) was observed in 2 patients, but control was maintained well in the other 4. Protein intake increased without hyperammonemia in 4 patients. The plasma level of isoleucine decreased in 4 patients, and supplement with essential amino acids was initiated in 2 of them. No adverse effects associated with NaPB were reported in this study.
Conclusion These results showed that NaPB is safe and effective in increasing protein intake without hyperammonemia. NaPB is used as a standard treatment for UCDs in many countries. This agent should be promptly approved for clinical use in Japan.
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Original Article
Title
Early Diagnosis of Kawasaki Disease in Patients Presenting with Only Fever and Cervical Lymphadenopathy
Author
Hiroshi Igarashi1)2) Hiroyuki Betsui1)2) Takane Ito1)2) Ritei Uehara3) Hirohiko Shiraishi2) and Mariko Momoi2)
1)Oyama Municipal Hospital
2)Department of Pediatrics, Jichi Medical University
3)Department of Public Health, Jichi Medical University
Abstract
The purpose of the present study was to differentiate Kawasaki disease accompanied by only fever and cervical lymphadenopathy (LKD) from bacterial lymphadenitis (BL) at initial presentation. We analyzed 22 patients who presented with only fever and mainly unilateral enlarged lymph node on admission to our hospital between January 2005 and June 2009. Of the 22 patients, 12 were rendered afebrile after antibiotic therapy and were classified as BL. The remaining 10 patients had other principal clinical signs of KD after the fourth day of illness and were classified as LKD; these patients were successfully treated with a single intravenous infusion of 1 g/kg or 2 g/kg of immunoglobulin.
There were no differences between the LKD and BL patients with regard to their age, size and local features of the lymph nodes, as determined by physical examination and ultrasonography. However, laboratory findings revealed a lower platelet count (29.6× 104 vs. 33.7× 104 /μl, median, p=0.016) and higher aspartate aminotransferase (AST) level (29 vs 23 IU/l, p=0.04) in the LKD patients than in BL patients between the second and fourth day of illness. Re-examination on the second or third day after antibiotic therapy revealed higher C-reactive protein (10.5 vs. 2.7 mg/dl, p< 0.001), AST (81.0 vs. 25.5 IU/l, p=0.009) and alanine aminotransferase (ALT) levels (45.5 vs. 11.0 IU/l, p=0.004) in the LKD patients than in BL patients. However, there was no difference in the platelet counts between the LKD and BL patients. When a cutoff point of platelet count on the day of hospitalization was set at 32× 104 /μl, we were able to distinguish LKD from BL with 80% sensitivity and 75% specificity.
Our results showed that the decrease in the platelet counts at the early stages preceded the appearance of other principal signs of KD, elevation of AST or ALT levels, and decrease of CRP levels; therefore the decrease in platelet count is an essential feature that would help in the diagnosis of LKD.
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Original Article
Title
Detection of E. coli O-74 in Fecal Samples from Five Patients with Kawasaki Disease
Author
Masahiko Okada Nobuo Honma Makiko Hayashi Makoto Satoh and Yuri Matsuda
Department of Pediatrics, Yonezawa City Hospital
Abstract
E. coli O-74 was detected in fecal samples obtained on admission from 5 patients with Kawasaki disease. The patients were 3 boys and 2 girls, aged from 2 to 4 years, who were admitted with high fever. The study was performed between July 2008 and August 2009. Each patient demonstrated high fever, but none received any antibiotics. Regarding gastrointestinal complaints, 4 patients had abdominal pain, 3 patients had abdominal distension, 2 patients had constipation, and all patients had either vomiting or diarrhea. After admission, all patients were diagnosed as having Kawasaki disease based on standard criteria. Four patients were treated with a high dose of gamma globulin and aspirin. One patient was treated with aspirin alone. None of the patients demonstrated significant progression of coronary artery abnormalities. Pathogenic E. coli O-74 was detected from stool cultures of these 5 patients who were not treated with antibiotics. Toxin production and adhesion test were also examined for 4 stored samples of stool from the 5 patients, but none of these were positive. We report the clinical course of these patients and discuss the role of E. coli O-74 in the onset of Kawasaki disease.
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Original Article
Title
A Case of Frontal Osteomyelitis and Epidural Abscess Developing as Complications of Acute Sinusitis
Author
Aya Goji Takanori Sekiguchi Natsuko Kishi Maki Shimizu Tomoko Takahashi Hiroko Kouzan Zenichi Sakaguchi and Katsuaki Ohara
Department of Pediatrics, Takamatsu Red Cross Hospital
Abstract
We report the case of a 14-year-old girl who suffered from prolonged fever, headache, and general malaise. An MRI performed the day before admission to our hospital, which was day 6 after the onset of fever, showed acute sinusitis without intracranial lesions. Since her symptoms continued unabated on day 7 in hospital, CT and repeat MRI examinations were performed, and frontal osteomyelitis and an epidural abscess were detected as complications of acute sinusitis.
Following intravenous administration of clindamycin and panipenem/betamipron, the patient underwent endoscopic surgery of the right nasofrontal duct. A silicon tube was placed in the duct for drainage. Surgical drainage of the epidural abscess was not performed. The patient was treated with intravenous antibiotics for 4 weeks. The frontal osteomyelitis and epidural abscess had disappeared on MRI images at 5 months after discharge. This was an unusual case of sinusitis complicated with frontal osteomyelitis and an epidural abscess that developed in just 1 week.
This case suggests that imaging tests should be performed repeatedly if symptoms continue unabated. In this case, it should be noted that surgical drainage of the epidural abscess was not performed. In fact, the patient showed a full recovery after the endoscopic paranasal sinus surgery, which has the advantage of causing less surgical trauma, and administration of antibacterial drugs for 4 weeks.
We believe that this case will be a useful reference for future treatments of cranial complications of sinusitis.
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