|
THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
|
Vol.123, No.9, September 2019
|
Original Article
Title
Efficacy and Safety of Urinastatin for Nephritis Prophylaxis in Pediatric Patients with IgA Vasculitis
Author
Yoshimitsu Gotoh1) Toshiki Kasagi1) Hisakazu Majima1) Katsuaki Kasahara1) Kazuki Tanaka2) Satoshi Hibino2) Naoya Fujita2) and Osamu Uemura3)
1)Department of Pediatric Nephrology, Japanese Red Cross Nagoya Daini Hospital
2)Department of Nephrology, Aichi Children's Health and Medical Center
3)Ichinomiya Medical Treatment & Habilitation Center
Abstract
IgA vasculitis (IgAV) is a systemic vasculitis characterized by purpura, abdominal pain, and arthralgia. The onset of Henoch-Schonlein nephritis (HSPN) primarily determines prognosis.
We have previously reported the efficacy of urinastatin in protecting against nephritis in IgAV patients at high risk for it. However, the evidence level was moderate. In this study, we examined urinastatin's safety and prophylactic efficacy against nephritis.
Using simple randomization, we distributed patients with primary-onset IgAV into urinastatin-administered (U) and non-administered (non-U) groups. Ultimately 50 patients (27 patients in the U group and 23 patients in the non-U group) were included in the study. There were no significant differences in background characteristics between the groups. The rate of nephritis was 18.6% (5 patients) in the U group and 17.4% (4 patients) in the non-U group. There was no significant difference between the two groups. We could not prove the efficacy of urinastatin in protecting against HSPN.
The risk score of developing HSPN was set at 50%. However, the rate of nephritis development in our study was lower than that reported in previous studies. This may be because many patients were undergoing concurrent aggressive therapy for focal infection.
|
|
Original Article
Title
Longitudinal Evaluation of Laboratory Findings during Parechovirus A Infection
Author
Eiki Ogawa1) Kensuke Shoji1) Masanori Kaneko2) Noriko Kawamura2) Shinji Oana3) Chitose Koyama-Wakai1) Noriyasu Iwase1) and Isao Miyairi1)
1)Division of Infectious Diseases, National Center for Child Health and Development
2)Department of Pediatrics, Kitasato University Hospital
3)Department of Pediatrics, Nishisaitama Chuo National Hospital
Abstract
Background: Numerous reports have described the clinical manifestations of parechovirus A (PeV-A) infection, but few reports have described the time course of laboratory findings during PeV-A infection.
Methods: Patients who were diagnosed with PeV-A infection on the basis of the results of a polymerase chain reaction test between April 2014 and September 2016 were enrolled. Laboratory findings and patient demographics were extracted from their medical records. The first day of the illness was defined as the day of onset of fever.
Results: Thirty-eight patients were enrolled. All the patients presented with fever, and 35 patients showed signs of circulatory failure. Leukopenia was observed in 26 patients (68%), which bottomed out at 3,570/mm3 (median) on day 3 of the illness. Elevated liver enzyme levels were found in 23 patients (61%), and the median peak levels for aspartate transaminase and alanine transaminase were 193 IU/L and 70 IU/L, respectively, on day 5 of the illness; The elevated liver enzyme levels decreased without treatment. The median peak value for the activated partial thromboplastin time was 62.9 seconds on day 6 of the illness. Vitamin K was administered to eight patients (21%); however, none of the patients had bleeding as a complication.
Conclusion: Leukopenia and liver enzyme level elevation were common in PeV-A infection and were resolved spontaneously without intervention.
|
|
Case Report
Title
Cardiac Dysfunction in a WPW Syndrome and Left Ventricular Non-compaction Patient with Chest Pain during Exercise Tolerance Test
Author
Nanami Oiki1) Shiro Baba1) Tomohiro Ozaki2) Kentaro Akagi1) Koichi Matsuda1) Daisuke Yoshinaga1) Takuya Hirata1) Hiroshi Katayama2) and Junko Takita1)
1)Department of Pediatrics, Kyoto University
2)Department of Pediatrics, Osaka Medical College
Abstract
WPW syndrome is a pre-excitation syndrome characterized by delta wave in ECG. Although the ECG changes is well known as an inducible factor of supraventricular tachycardia (SVT), most of the patients do not have any symptoms nor arrhythmia. However, differential diagnosis is sometimes needed because ECG shows pseudo-ventricular tachycardia and ST change during SVT.
We diagnosed WPW syndrome in a patient with chest pain and ST change in ECG during exercise as left ventricular non-compaction with cardiac dysfunction by echocardiography. Assessed by various examinations, catheter ablation was performed for the first therapy. Although the cardiac dysfunction improved by ablation, the improvement of cardiac contraction was not dramatic. This implies not only WPW syndrome but also the complication of left ventricular non-compaction influence on left ventricular function. Thus, further precise follow-up for cardiac function is indispensable for this patient.
|
|
Case Report
Title
Episodic Ataxia Type 2 with a Novel Missense Mutation of CACNA1A Gene
Author
Nozomu Ozaki
Kadono Sanjo Children's Clinic
Abstract
Episodic ataxia type 2 (EA2) is a rare disease characterized by transient recurrent ataxia, which is often triggered by physical exertion. EA2 is defined by the detection of mutations of CACNA1A which encodes the pore-forming and voltage-sensing α1A subunit of a neuronal voltage-gated calcium channel, Cav2.1 P/Q-type calcium channel.
In this case of EA2 in an 11-year-old boy, he experienced periodic attacks of falling down soon after abrupt staggering since the age of 1 year and 7 months. Attacks occurred subsequent to running around, and lasted 2 to 3 hours. Although several anti-epileptic drugs were given, falling attacks were not suppressed. Between attacks he showed mild truncal ataxia and abnormal eyeball movement. After the introduction of an acetazolamide treatment (500 mg per day) at 10 years of age, he experienced no attack during the follow up period of 1 year. Apart from this, he experienced generalized tonic-clonic convulsions during fever 10 times since the age of 11 months until 30 months.
Genetic testing revealed a missense CACNA1A mutation (c. 5373T>G p.Phe 1791 Leu). This mutation is novel and is not listed on dbSNP.
|
|
Case Report
Title
Difficulty in Differentiating a Diagnosis of Disease Relapse from Focal Nodular Hyperplasia in Advanced Neuroblastoma: A Report of Two Cases
Author
Kayo Ochiai1) Ai Yamada1) Mariko Kinoshita1) Daisuke Sawa1) Yusuke Saito1) Sachiyo Kamimura1) Yuichiro Sato2) Takuro Nishikawa3) Yasuhiro Okamoto3) Yoshifumi Kawano3) Masato Kawano4) Takafumi Kawano4) Satoshi Ieiri4) and Hiroshi Moritake1)
1)Division of Pediatrics, University of Miyazaki Hospital
2)Department of Diagnostic Pathology, University of Miyazaki Hospital
3)Department of Pediatrics, Kagoshima University Hospital
4)Department of Pediatric Surgery, Kagoshima University Hospital
Abstract
Focal nodular hyperplasia (FNH) is a benign tumor of the liver that is rare during childhood; however, the incidence has been increasing among patients with malignancy who have been treated with chemotherapy, radiotherapy, and hematopoietic stem cell transplantation (HSCT). The therapeutic principle of FNH is a conservative "wait and see" policy; however, FNH shows various imaging findings, especially when patients have a history of intensive therapy. Therefore, it is difficult to discriminate FNH from local recurrence or metastasis when images show a hypervascular pattern. We herein report two patients with FNH who were previously treated with comprehensive therapy, including HSCT for neuroblastoma. In both cases, it was difficult to exclude the possibility of relapse or new malignancies; therefore, surgical resection was performed. Eventually, FNH in both cases was diagnosed by a pathological examination. FNH should be included as a differential diagnosis for patients with a history of intensive therapy who develop hepatic tumors.
|
|
Case Report
Title
Three Cases of Drug-induced Hypersensitivity Syndrome Diagnosed by the Reactivation of HHV-6
Author
Taisuke Ono Keiji Yonekura Keiichi Hara Yasuhiko Sera and Shinichiro Miyagawa
Department of Pediatrics, National Hospital Organization KURE Medical Center
Abstract
Drug-induced hypersensitivity syndrome (DIHS) is a late-onset drug eruption and is associated with the reactivation of human herpesvirus-6 (HHV-6). Early diagnosis and therapy are important because patients usually have a fatal course.
We report three cases of DIHS diagnosed on the basis of clinical symptoms and virological examination findings.
Case 1 was a patient who had a history of Kawasaki disease. Treatment was started based on an initial diagnosis of Kawasaki disease reactivation; however, his symptoms failed to improve, and he developed pulmonary edema. We diagnosed DIHS caused by aspirin on the basis of detection of HHV-6 by separating the virus. Case 2 was a patient referred to our hospital for suspected measles who was diagnosed with DIHS and treated accordingly. DIHS was diagnosed because we detected HHV-6 by separating the virus and patient gave a history of diaminodiphenyl sulfone (DDS) use for acne. Case 3 was a patient referred to our hospital for suspected infectious mononucleosis. He was diagnosed with DIHS due to elevated HHV-6 IgG levels in paired sera and a history of DDS use for acne and was treated accordingly.
There are many diseases in which fever and eruption are the main symptoms, and we have to consider DIHS as a possible cause. On suspicion of DIHS, the suspected offending drug must be discontinued and the patient must be tested for the reactivation of HHV-6.
|
|
Case Report
Title
A 1-year-old Girl with Recurrent Nephrotic Syndrome Following Vaccinations against Varicella and Influenza
Author
Hiroaki Kanai1) Emi Sawanobori2) Hiroki Sato1) and Yoshichika Takei1)
1)Department of Pediatrics, Suwa Central Hospital
2)Department of Pediatrics, Faculty of Medicine, University of Yamanashi
Abstract
A 1-year-old girl developed nephrotic syndrome (NS) and achieved remission following administration of prednisolone (PSL), with subsequent tapering for 8 weeks. Twenty days after termination of PSL, a second varicella vaccine was administered. Proteinuria appeared 7 days later, and recurrent NS was diagnosed. She achieved remission on day 12 of PSL re-administration, followed by dose tapering. Although the patient received an initial influenza vaccine 10 days after PSL dose reduction, proteinuria was detected 4 days later. Subsequently, she was again diagnosed with recurrent NS and treated with PSL and entered remission on day 7. The PSL dose was tapered and discontinued after a total of 2 months. As proteinuria developed twice within a single week following vaccination and was not transient, vaccination was determined to be the trigger inducing recurrence. Although she received a 4-antigen combination vaccine and an influenza vaccine 4 times (5 vaccinations in total), 6 months after cyclosporine initiation, there was no relapse; therefore, the timing of vaccination and the concomitant immunosuppressants were believed to have prevented NS relapse. Although there is no clear causal relationship, vaccination can increase the likelihood of NS relapse, especially shortly after remission. Caution is required for at least the first 2 weeks following vaccination, regardless of the type of vaccine, and frequent home urinalysis is recommended.
|
|
Case Report
Title
A Case of Kurdish Biotinidase Deficiency Not Detected by C5-OH Elevation in Newborn Screening
Author
Keisuke Okada1) Sayaka Ajihara1) Ikuma Musha1) Masato Arao1) Tomomi Naito3) Kumiko Inoue3) Shoichi Oyama3) Kei Murayama4) Yasushi Okazaki5) Toru Kikuchi1) Ken-ichi Tokuyama1) and Akira Ohtake1)2)
1)Department of Pediatrics, Saitama Medical University
2)Center for Intractable Diseases, Saitama Medical University Hospital
3)Department of Pediatrics, Saiseikai Kawaguchi General Hospital
4)Department of Metabolism, Chiba Children's Hospital
5)Intractable Disease Research Center, Graduate School of Medicine, Juntendo University
Abstract
A one-month-old girl came to our hospital because of repeated seizures. Newborn screening (NBS) results had been normal, including those for 3-hydroxy-isovalerylcarnitine (C5-OH). The parents were Kurdish and first cousins. Her older brother died due to respiratory problems at 3 days of age. Her brain T2-weighted magnetic resonance imaging showed a high signal at the bilateral internal capsule. We detected an elevated C5-OH level of 1.52 nmol/mL (cut off <1.0 nmol/mL) in the blood on filter paper using mass spectrometry (MS)/MS as well as elevated 3-hydroxyisovaleric acid and lactate levels in the urine using gas chromatography (GC)/MS. Elevated plasma lactate and pyruvate levels as well as increased lactate-to-pyruvate ratio were also found. Under the clinical diagnosis of Leigh syndrome, both biochemical and genetic analyses were done. Whole-exome analysis revealed that she had the homozygous c.98-104del7ins3 mutation in the biotinidase gene. After the start of high-dose biotin, her symptoms gradually improved except her hearing difficulty. In Japanese NBS, the elevation of C5-OH levels is an indicator of biotinidase deficiency. However, our case shows that C5-OH is irrelevant. The measurement of biotinidase activity itself must be considered to detect biotinidase deficiency accurately in NBS.
|
|
Case Report
Title
Acute Pulmonary Injury Due to Inhalation of a Waterproof Spray in a Mother and Her Child
Author
Yumiko Ikemoto Eriko Kitera Yoshiko Taninaka and Tomoko Kawata
Department of Pediatrics, Himeji St. Mary's Hospital
Abstract
The patients were a 34-year-old female and her 4-year-old son. They started to cough 30 minutes after inhalation of a waterproof spray and developed dyspnea 10 hours later. The adult female had used the spray in a room. The patients were admitted to our hospital on day 2 after the incident.
The male child had hypoxemia, which required oxygen therapy for three days. On chest radiography, there were ill-defined areas of increased density in both the bilateral middle and lower lobes. On day 6, the symptoms were relieved, and on day 14, the chest X-ray findings returned to normal. The mother did not have hypoxemia. However, her chest X-ray showed ill-defined areas of increased density in the right lung and both the middle and lower lobes of the left lung. In addition, her computed tomography scan showed extensive bilateral areas of ground-glass opacities with interlobular septal thickening. On day 4, her dyspnea was relieved, and on day 8, her chest X-ray showed resolution of the increased density. Both patients recovered satisfactorily following treatment with antibiotics and steroids without any residual pulmonary sequelae.
Inhalation of waterproof spray can cause extensive pulmonary injury. Although the dangers involved are not fully understood, waterproof spray is commercially available. Further efforts are needed to make their potential health risks publicly known.
|
|
Case Report
Title
A Diagnostic Pitfall in an Elementary School Check-up Using Growth Charts in a Girl with Primary Hypothalamus Brain Tumor
Author
Tatsuki Urakawa1) Satoshi Watanabe1) Nobuhiro Ito1) Shinji Tanioka1) Yasutomo Funakoshi1) Tetsuya Hirota2) Eiichi Kinoshita3) Hiroyuki Moriuchi1) and Sumito Dateki1)
1)Department of Pediatrics, Nagasaki University Hospital
2)Hirota Children's Clinic
3)Kinoshita Children's Clinic
Abstract
Brain tumors in or near the pituitary gland and the hypothalamus in childhood often show various clinical symptoms including growth disruption. We report a 9-year-old girl with hypothalamus brain tumor and reduced growth rate. She presented with headache and vomiting and was admitted on an emergency basis to our hospital because of prolonged hypoglycemia. We diagnosed hypothalamic germinoma complicated with panhypopituitarism, based on brain CT scan findings, increased levels of tumor markers, low serum cortisol level during a state of hypoglycemia, and central hypothyroidism. A review of her growth chart indicated that her height growth rate had decreased since 3 years previously. In addition, she started to complain of polydipsia and polyuria 4 years ago. Although growth charts have been used for health management of elementary and junior high school students in Japan since April 2017, this management system failed to detect the decreased growth rate of the patient, partly because height data before entering the elementary school were not plotted on the growth chart. Not only growth data at the elementary school but also those during the preschool period are needed to screen the diseases that affect growth rates, especially in lower-grade elementary school pupils.
|
|
Brief Report
Title
Attitudes of Parents toward Cancer Education in School
Author
Koshu Sugisaki
Niigata University of Health and Welfare
Abstract
Cancer education in school is becoming implemented in earnest. It is necessary to gain understanding of the parents to enhance the cancer education. However, attitudes of parents toward cancer education have not been clarified so far. In this study, a nationwide survey was conducted in August 2018, and 2,417 parents of elementary, junior high, and high school students responded. Only 11% of parents were aware that cancer education would be conducted at school, but about 73% of parents agreed that cancer education should be offered at school. Moreover, respondents believed that about 70% or more of all contents in cancer education is necessary or somewhat necessary. This study clarified parental favor of cancer education in school that consists of varies contents. Thus, it needs to be enhanced through the cooperation of doctors (school doctors, cancer specialists, etc.), cancer survivors, cancer institutional hospital officials, and others.
|
|
Brief Report
Title
Disaster Medical Management for Children and Pregnant Women by the PPDML (Pediatric and Perinatal Disaster Medical Liaison) Related to the North Osaka Earthquake 2018
Author
Shinsuke Furuya1)2) Tomomi Yoshino1) Junichi Ishikawa1) Hiroshi Rinka1) Kimiko Yamashita2) Kazuharu Tanaka2) Muneyuki Takeuchi2) Kiyoshi Takemoto2) Kazuhide Ogita2) Hiroyuki Ichiba2) Kazuko Wada2) and Miho Misaki3)
1)Emergency and Critical Care Medical Center, Osaka City General Hospital
2)Pediatric and Perinatal Disaster Medical Liaison Osaka
3)Department of Clinical Research, National Disaster Medical Center
Abstract
Children and pregnant women are vulnerable and need much support in disasters. On the occasion of the North Osaka Earthquake 2018, the PPDML started coordination with DMAT (Disaster Medical Assistance Team) in disaster medical headquarters. We coordinated transport of 22 pediatric patients from damaged hospitals. Because PPDML had been praticed in disaster drills before, the experience could allowed PPDML to achieve good performance in real disasters. We conclude that routine cooperation between disaster medical networks and PPDML is essential not only in disasters but in general clinical work.
|
|
|
Back number
|
|
