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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.119, No.4, April 2015


Original Article
1. The Immunogenicity and Safety of a New Quadruple Combination Vaccine, Including Undiluted IPV (Salk vaccine)
2. TmP/GFR is a More Useful Marker than %TRP in Making a Clinical Diagnosis of X-linked Hypophosphatemic Rickets Caused by the PHEX Gene Mutation
3. Awareness Survey Concerning Pediatric Emergency Telephone Consultation in Osaka among Emergency Departments Visited Nocturnally
Case Report
1. A Case of Infantile Idiopathic Pulmonary Arterial Hypertension (iPAH) with Syncope
2. Two Cases of Secondary Bacteremia after Rotavirus Gastroenteritis
3. Focal Nodular Hyperplasia of the Liver Associated with a Congenital Extrahepatic Portosystemic Shunt Discovered in Early Adolescence


Original Article
Title
The Immunogenicity and Safety of a New Quadruple Combination Vaccine, Including Undiluted IPV (Salk vaccine)
Author
Tetsuo Nakayama1) Hideyuki Yamauchi2) Ortiz Esteban3) Valérie Bosch Castells3) and Satoshi Nishina4)
1)Kitasato Institute for Life Sciences, Kitasato University
2)Daiichi Sankyo Co., Ltd.
3)Sanofi Pasteur S. A.
4)Sanofi K. K.
Abstract
The immunogenicity and safety of a new quadruple combination vaccine (DTaP-IPV) manufactured by combining undiluted DTaP (Kitasato Daiichi Sankyo Vaccine Co., Ltd.) with undiluted IPV (Salk vaccine) (Sanofi Pasteur) were investigated.
A total of 377 Japanese children were randomized into a DTaP-IPV group (Group A) and DTaP+OPV group (Group B) at a 2:1 ratio. A three-dose subcutaneous primary vaccination with DTaP-IPV for Group A and DTaP for Group B was followed by a single-dose booster vaccination. Subsequently, a two-dose oral administration of OPV placebo or OPV was given to Groups A and B, respectively.
The seroprotection rates against poliovirus types 1, 2, and 3 at 1 month after primary vaccination were all 100% in Group A, and the non-inferiority of DTaP-IPV versus DTaP+OPV in terms of seroconversion rates for all antigens was demonstrated. In addition, the seroconversion rates for poliovirus types 1, 2, and 3 at 1 month after booster vaccination were all 100% in Group A, with a marked increase in GMTs for all antigens.
The incidence of adverse reactions was comparable in group A and group B, with no significant difference in incidence of injection site reactions or fever between both groups. These results suggest that DTaP-IPV is highly immunogenic and not associated with significant safety concerns in Japanese children. (Japic CTI number: JapicCTI-101350)




Original Article
Title
TmP/GFR is a More Useful Marker than %TRP in Making a Clinical Diagnosis of X-linked Hypophosphatemic Rickets Caused by the PHEX Gene Mutation
Author
Ryojun Takeda1) Kentaro Miyai1) Masaki Takagi1) Masahiro Goto1) Daisuke Ariyasu2) Masako Izawa3) Junko Igaki4) Eri Suzuki5) Yoshie Nakamura6) and Yukihiro Hasegawa1)6)
1)Division of Endocrinology and Metabolism, Tokyo Metropolitan Children's Medical Center
2)Division of Developmental Genetics, Institute of Resource Development and Analysis, Kumamoto University
3)Department of Pediatric Endocrinology and Metabolism, Aichi Children's Health and Medical Center
4)Division of Endocrinology and Metabolism, Gunma Children's Medical Center
5)Department of Pediatrics, National Hospital Organization Tokyo Medical Center
6)Division of Genetic Research, Tokyo Metropolitan Children's Medical Center
Abstract
The clinical diagnosis of X-linked hypophosphatemic rickets (XLH) is based on a number of biochemical observations. These include a reduction in the percentage of tubular reabsorption of phosphate (%TRP), and in the maximal tubular phosphate reabsorption capacity corrected for glomerular filtration rate (TmP/GFR). However, it is important to maintain sufficient renal blood flow in order to accurately calculate TmP/GFR.
The aims of this study were to compare the normal reference values of %TRP and TmP/GFR with the values detected in XLH patients with and without pre-examination water loading, and to compare the time-dependent alterations in %TRP and TmP/GFR values in XLH patients who underwent water loading either from the beginning of the test or the day before testing.
1) In the analysis of %TRP and TmP/GFR, calculated in 286 samples from 26 XLH patients without water loading, 64 samples for %TRP and 4 samples for TmP/GFR showed values within the reference ranges. 2) A time-dependent decrease in %TRP and TmP/GFR was observed in cases of XLH patients who performed water loading from the beginning of the test. However, time-dependent alterations in %TRP and TmP/GFR were not identified in patients who underwent water loading from the day before testing. 3) In the analysis of %TRP and TmP/GFR, calculated in 48 samples from 22 XLH patients after water loading, 25 samples for %TRP showed values within the reference range, and all samples for TmP/GFR showed values lower than the reference value.
TmP/GFR is a more useful marker than %TRP in making a clinical diagnosis of XLH caused by the PHEX gene mutation. Furthermore, it is important to maintain sufficient renal blood flow in order to accurately calculate TmP/GFR.




Original Article
Title
Awareness Survey Concerning Pediatric Emergency Telephone Consultation in Osaka among Emergency Departments Visited Nocturnally
Author
Masako Fukui1) Urara Koudera1)2) Takehisa Yamamoto1)3) Shinji Katagiri1)4) Hiroko Fujitani1) Tetsuhisa Takechi1) and Minoru Ogawa1)
1)Osaka Pediatric Association
2)Department of Pediatrics, Nakano Children's Hospital
3)Department of Pediatrics, Minoh City Hospital
4)Katagiri Children's Clinic
Abstract
Since 2008, pediatric emergency telephone consultations in Osaka (#8000), have averaged more than 100 per day. To determine the popularity and the role of this service, we conducted a questionnaire survey targeting nocturnal pediatric emergency department visits. According to the survey, 433 of 644 visitors (67%) were aware of the consultation service, and 460 of 644 (71%) were eager to use it when necessary. Consultations on whether to visit the outpatient clinic or not accounted for 70% of the telephone calls. Of all nocturnal outpatient visitors, 16% visited hospitals before making use of the phone. They tended to use the internet less often and assumed that they had no other choice than to directly seek medical attention, which should be taken into account when considering nighttime pediatric healthcare systems.
The reason of "I think my child has a bad symptom" accounted for 70% of the reasons for visiting an emergency department during the night, and "I was advised to visit a hospital on the phone" accounted for 6%. The latter most often required hospitalization, and only a few of them underwent only physical examination or drug administration. We found many cases in which patients stated that they would use the phone, but date of the event, they sought medical attention rather than make the phone call. Thus, it is important to establish simple standards by which patients themselves can decide whether they should visit an outpatient clinic according to the symptoms.




Case Report
Title
A Case of Infantile Idiopathic Pulmonary Arterial Hypertension (iPAH) with Syncope
Author
Kenji Sugamoto1) Kiyoshi Ogawa1) Kenji Hoshino1) Takashi Hishitani1) Yukinori Saito1) Yoshitaka Fujimoto1) Seiichi Kagimoto2) Mitsuru Kubota2) and Shinichiro Hagiwara2)
1)The Department of Cardiology, Saitama Children's Medical Center
2)The Department of General Pediatrics, Saitama Children's Medical Center
Abstract
We report a case of infantile idiopathic pulmonary arterial hypertension (iPAH). The patient presented with cyanotic syncope when crying at the age of two months. Between cyanotic attacks, she had no signs of congestive heart failure or cyanosis, and an ultrasound cardiogram revealed no evidence of pulmonary hypertension. However, cardiopulmonary resuscitation had been required at the time of severe cyanotic attacks. The ultrasound cardiogram did reveal severe tricuspid regurgitation and a right-to-left directional intra-atrial shunt through the foramen ovale during severe cyanotic attacks. Cardiac catheterization under deep sedation revealed moderate pulmonary hypertension. Oral pulmonary vasodilative medications were not effective for controlling the cyanotic attacks. Repeat cardiac catheterization showed extreme elevation of the pulmonary arterial pressure, which was induced by awakening. Because the cyanotic attacks were uncontrolled, continuous intravenous infusion of prostaglandin I2 (PGI2) was started. After PGI2 administration, her cyanotic attacks diminished. The pathological findings of a lung biopsy were a thickened media of the small pulmonary arteries and hypoplasia of pulmonary arteries smaller than 150 μ m in diameter. PGI2 administration was considered to be effective for the symptoms of infantile iPAH, which was based on the spasm of the pulmonary vasculature.




Case Report
Title
Two Cases of Secondary Bacteremia after Rotavirus Gastroenteritis
Author
Mioko Kasagi1) Tasuku Takadera1) Tatsuya Nishimura1) Ken Matsubara1) Kenji Morimoto1) Yukie Arahata1) Hironobu Kobayashi1) Masayoshi Senda1) Katsuhiko Kitazawa1) Akihito Honda1) and Masashi Kobayashi2)
1)Department of Pediatrics, Asahi General Hospital
2)Division of Pediatric Critical Care, Shizuoka Children's Hospital
Abstract
We report 2 cases of rotavirus gastroenteritis complicated by enteric gram-negative bacillus (GNB) bacteremia. Case 1 was a previously healthy 7-month-old boy who was admitted because of watery diarrhea and vomiting for 5 days. On the fifth day of admission, he had a high fever. His blood tests showed white blood cells (WBC) of 13,100/μl and C-reactive protein (CRP) of 14.81 mg/dl. A blood culture grew a GNB subsequently identified as Klebsiella pneumoniae. He was successfully treated with cefotaxime. Case 2 was a 3-year-old girl with Mayer-Rokitansky-Kuster- Hauser syndrome who was admitted because of severe diarrhea. On the fifth day of admission, she suddenly developed a high fever. Laboratory examinations showed WBC of 9,900/μl, CRP of 0.68 mg/dl and normal urine analysis. Considering the risk of urinary tract infection, cefotaxime and ampicillin were started. A blood culture yielded Escherichia coli but a urine culture was negative. She was discharged in good health after completing antibiotic therapy. In both cases, stool samples were positive for rotavirus antigen on admission. We speculate that translocation of enteric flora across the intestinal epithelium damaged by rotavirus might play a role in developing bacteremia. Secondary bacteremia after rotavirus gastroenteritis could occur in infants and young children without any specific medical histories, and a fatal case has also been reported. We should keep this rare but dangerous complication in mind during the course of rotavirus gastroenteritis. It is important to obtain blood cultures and to start effective antibiotics for enteric GNB when a child with rotavirus gastroenteritis develops secondary fever.




Case Report
Title
Focal Nodular Hyperplasia of the Liver Associated with a Congenital Extrahepatic Portosystemic Shunt Discovered in Early Adolescence
Author
Genrei Ohta1) Tomomi Yoshino1) Ikue Hata1) Kouichi Mizuta2) and Yusei Ohshima1)
1)Department of Pediatrics, Faculty of Medical Sciences, University of Fukui
2)Department of Transplant Surgery, Jichi Medical University
Abstract
Focal nodular hyperplasia (FNH) of the liver is a rare benign tumor-like lesion and is occasionally associated with congenital extrahepatic portosystemic shunt (CEPS). A 12-year old boy complained of right back pain. Laboratory findings revealed elevated serum γ-GTP levels, and an abdominal echogram showed multiple nodular lesions in the liver. Imaging studies diagnosed the lesions as FNH associated with CEPS. Because of abnormal MRS signals in the basal ganglia, which suggested chronic hyperammonemia and an increased intrapulmonary shunt follow rate, he received living donor liver transplantation from his father. After transplantation, the back pain disappeared, and the abnormal findings in the brain MRI ameliorated. FNH and underlying CEPS should be considered as a differential diagnosis for liver tumors. Liver transplantation should be indicated to avoid irreversible higher brain dysfunction and portopulmonary hypertension.




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