gakkaizashi


THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.120, No.11, November 2016


Original Article
1. The Outcome of Neonates with Bilious Vomiting at a Single Institution
2. Current Utilization Status of Medical Information Notebook for Caregivers of Medically Complicated Children with Special Health Care Needs
3. End-of-life Care for Children with Brain Tumors: Eleven-year Experience at Nagasaki University Hospital
4. The Coverage of Haemophilus influenzae Type b and 7-Valent Pneumococcal Conjugate Vaccines in Japan: Impact on the Incidence of Pediatric Bacterial Meningitis in Kanagawa from 2008 to 2013
5. Analysis of 11 Pediatric Cases of Brain Abscess
Case Report
1. Recovery of Central Diabate Insipidus Induced by a Rathke's Cleft Cyst
2. A Case of Vitamin D Deficiency Rickets Caused by Feeding Problems with Autism Spectrum Disorder
3. A Case of Severe Combined Immunodeficiency with Vaccine-acquired Rotavirus
4. Zinc Deficiency in Low Zinc Breast Milk Related to Maternal ZnT2 Gene Mutation
5. A Boy of Polyarteritis Nodosa with Coronary Artery Lesion Who Attained Remission with Plasma Exchange and High Dose Glucocorticoid Therapy


Original Article
Title
The Outcome of Neonates with Bilious Vomiting at a Single Institution
Author
Minori Tamai1)2) Atsushi Nemoto1) Ken Takada1) Mami Kobayashi1) Takayuki Komai1) Kanji Sugita2) and Atsushi Naito1)
1)Division of Neonatology, Perinatal Center, Yamanashi Prefectural Central Hospital
2)Department of Pediatrics, Yamanashi University Hospital
Abstract
Bilious vomiting is an important symptom in neonates indicating digestive malfunction and sometimes requiring surgical intervention. The outcome of neonates with bilious vomiting has been reported in some countries, but not throughout Japan. We conducted a retrospective review of the medical records in our institution from 2006 to 2015 to clarify characteristics of neonates with bilious vomiting. A total of 24 neonates were divided into 8 neonates with exterior hospital birth and 16 with interior hospital birth. The time to first bilious vomiting episode was 2.5 days in average, ranging from day 0 to 8. Thirteen neonates (54%) required abdominal operation. This rate was higher than that reported previously in other countries. Episodes of bilious vomiting were more than twice in 15 neonates, only once in 7 neonates, and unknown in 2 neonates. Surgical intervention was required in 12 of 16 neonates (80%) who had bilious vomiting more than twice, but not in 8 neonates who had bilious vomiting only once. Therefore, it is important to consider surgical cases when neonates had bilious vomiting more than twice. For predicting prognosis, our present study might be useful.




Original Article
Title
Current Utilization Status of Medical Information Notebook for Caregivers of Medically Complicated Children with Special Health Care Needs
Author
Nobuyuki Yotani1)2) Yuji Iwasaki3) Michio Fukumizu4) Naoyuki Tanuma4) Sunao Tomita5) Sui Sone6) Hiroshi Ozawa7) Osamu Fukatsu4) Tomoo Nakamura1) Akira Ishiguro1) and Hirokazu Sakai1)
1)Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development
2)Department of Palliative Medicine, Kobe University Graduate School of Medicine
3)Tokyo Metropolitan Tobu Medical Center for Persons with Developmental/Multiple Disabilities
4)Tokyo Metropolitan Fuchu Medical Center for the Disabled
5)Tokyo Metropolitan Children's Medical Center
6)Tokyo Metropolitan Higashiyamato Medical Center for Developmental/Multiple Disabilities
7)Shimada Rehabilitation Center Hachioji
Abstract
A multidisciplinary panel (six pediatricians, one nurse and one physiotherapist) created a medical information notebook entitled the "Nagomi Notebook", for caregivers of medically complicated children with special health care needs (CSHCN). The notebook comprises three sections: medical information, rehabilitation programs and details of daily care. The purpose of this study was to elucidate the current status of utilization of the Nagomi Notebook. CSHCN and their families were recruited from two children's hospitals, one university hospital and six rehabilitation centers. The notebook was distributed to families with CSHCN at enrollment in 2011. One year later, the families received a self-reporting questionnaire. The analysis included 80 medically complicated CSHCN and their families. The mean age of the CSHCN was 15.9±12.1 (SD) years. The rates of those who were carrying the notebook was 46%. Among the CSHCN who visited the emergency room more than three times in the last six months, 75% stated that the medical information section of the notebook was useful. Using short-stay hospital services was positively correlated utilizing the Nagomi Notebook in all sections. (medical section: odds ratio [OR] =3.35, p=0.04; rehabilitation section: OR=2.91, p=0.09; care section: OR=2.89, p=0.08). We believe that it is more effective to distribute the Nagomi Notebook only to targeted caregivers of CSHCN.




Original Article
Title
End-of-life Care for Children with Brain Tumors: Eleven-year Experience at Nagasaki University Hospital
Author
Yasutomo Funakoshi Tasuku Kitajima Nobuhiro Ito Masahiko Okada and Hiroyuki Moriuchi
Department of Pediatrics, Nagasaki University Hospital
Abstract
Background: The mortality rate of brain tumors is relatively high among pediatric malignancies. Providing end-of-life care for pediatric patients with brain tumors can be difficult, as they tend to progress rapidly. We reviewed a variety of problems regarding end-of-life care among pediatric cases by analyzing our own patients.
Methods: A retrospective chart review was performed for 14 cases of brain tumors treated at the Department of Pediatrics, Nagasaki University Hospital, from April 2004 to April 2015.
Results: The age groups at the time of death were 0-5 years for 4 cases, 6-15 years for 8, and 16-24 years for 2. Nine and five were male and female, respectively. All of the cases were considered incurable at the time of relapse or progression. The periods from diagnosis to relapse or progression were less than one year in seven cases, one to less than three years in four, and three years or longer in three. The periods from relapse or progression to a decrease in consciousness were less than three months in eight cases. The periods from a decrease in consciousness to death were less than three months in all eight cases not using mechanical ventilation, whereas three of the six cases using mechanical ventilation survived for longer than three months and died at home, supported by home medical care.
Conclusions: Families should receive earlier discussions about end-of-life care, as their children may have only a few months to live after relapse or progression. Only three families were present at their child's final moment at home. More local medical resources as well as regional cooperation between medical institutions are needed to provide appropriate medical management and end-of-life care at home for children with brain tumors.




Original Article
Title
The Coverage of Haemophilus influenzae Type b and 7-Valent Pneumococcal Conjugate Vaccines in Japan: Impact on the Incidence of Pediatric Bacterial Meningitis in Kanagawa from 2008 to 2013
Author
Ryo Niiya1) Kanae Takahashi2) Masaaki Mori3) Tadaomi Tokutake4) Rei Fumimoto1) Takumi Moriuchi1) Ayano Shinagawa1) Yusuke Miyaji1) Yukitsugu Nakamura1) and Tomohiro Katsuta1)
1)Department of Pediatrics, St. Marianna University School of Medicine
2)St. Therese Chiisaki Hanano Sono
3)Department of Pharmacovigilance, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
4)Department of Pediatrics, Shonan-East General Hospital
Abstract
In Japan, Haemophilus influenzae type b (Hib) vaccine and 7-valent pneumococcal conjugate vaccine (PCV7) were licensed in 2008 and 2010, respectively. To evaluate the effectiveness of Hib vaccine and PCV for children, we conducted a retrospective questionnaire survey in various hospitals in Kanagawa from 2008 to 2013.
Hib vaccine and PCV7 were available in Japan from 2008 to 2010, but were not included in the National Immunization Program (NIP). Both vaccines were categorized as voluntary, requiring individuals seeking to be vaccinated to pay for the vaccines themselves. Therefore, coverage of both vaccines was low and supply factor was approximately 30-50%. Consequently, the incidence of bacterial meningitis did not decrease. Although remaining voluntary, in 2011 Hib and PCV7 vaccines were available to the public free of cost and the supply factor increased to approximately 120%. As a result, vaccine coverage increased and incidence of Hib meningitis was reduced from 23 to 6 cases, and pneumococcal meningitis from 10 to 6 cases respectively between 2010 and 2011. Finally, in 2013, both vaccines were included in the NIP.
Findings suggest that providing Hib vaccine and PCV free of cost was crucial to improving coverage rate.




Original Article
Title
Analysis of 11 Pediatric Cases of Brain Abscess
Author
Tadashi Hoshino1) Chie Fukasawa1) Shoko Tokutake1) Hideyuki Okui1) and Hiromichi Nakajima2)
1)Division of Infectious Diseases, Chiba Children's Hospital
2)Division of Cardiology, Chiba Children's Hospital
Abstract
We reviewed 9 pediatric patients with brain abscesses who were admitted to Chiba Children's Hospital between January 2001 and June 2015. Among these patients, 7 were boys and 7 had predisposing conditions; 6 had cyanotic congenital heart diseases and 1 had sinusitis. Two patients had recurrence, one time each. Including these cases, a total of 11 cases were enrolled in this study. The median age at onset was 4 years and 3 months. Five patients were aged less than 3 years. Neurological findings were observed in 9 of the 11 cases, and focal neurological signs in 4 cases. However, because 20 of the 36 clinical symptoms were nonspecific, such as fever and headache, the time between onset and diagnosis tended to be long (median, 6 days). Abscesses were observed in cerebral or cerebellar parenchyma in 10 patients, and in the lateral ventricle in 1 patient. Frontal and parietal lesions were most common, occurring in 3 patients each. Two abscesses were detected in 2 patients. Fifteen bacterial pathogens were isolated from 9 patients, with the Streptococcus anginosus group being the most commonly detected (8 strains). Polymicrobial pus was identified in 4 cases. All patients were given carbapenems as the initial antimicrobial therapy, and 7 of them were switched to target therapy after the detection of bacterial pathogens. Nine patients received surgical therapy. Eight patients whose abscess diameters were ≥2 cm in the parenchyma underwent drainage with small craniotomy. One patient with a lesion in the lateral ventricle underwent external ventricular drainage. Recurrence was observed in 2 patients, neurological sequelae in 3, and death in 1. No guidelines exist for the management of pediatric brain abscesses, and establishment of these guidelines is needed.




Case Report
Title
Recovery of Central Diabate Insipidus Induced by a Rathke's Cleft Cyst
Author
Eri Nakatani Katsuyuki Matsui Yoshihiro Maruo and Yoshihiro Takeuchi
Depertment of Pediatrics, Shiga University of Medical Science
Abstract
Here we report a case of a 11-year old girl with curable central diabetes insipidus (CDI) induced by a Rathke's cleft cyst. Three days before admission to the hospital, she had severe headache, vomiting, and polyuria. Magnetic resonance imaging revealed a hypophyseal cystic tumor. She was admitted to our hospital for examination and tumor enucleation. Although polyuria disappeared on the 4th admission day, it recurred with headache and fever on the 7th admission day. She was treated with hydrocortisone therapy for hypophysitis induced by self-destruction of the Rathke's cleft cyst. Although the headache and fever were cured, polyuria continued. We diagnosed CDI and central hypothyroidism. Thereafter, desmopressin acetate and thyroxin replacement therapy were initiated. Tumor enucleation was canceled because the Rathke's cleft cyst became too small to be safely operated upn. We guessed that her CDI would continue through life because there was no cured patient report of CDI caused by Rathke's cleft cyst without enucleation. However, it resolved about six months later and we could discontinue desmopressin acetate therapy. Hypothyroidism was also improved. CDI and hypothyroidism have never recurred since then. In our case, self-destruction of the Rathke's cleft cyst and hydrocortisone therapy for hypophysitis were considered to be responsible for the recovery of CDI induced by the Rathke's cleft cyst.




Case Report
Title
A Case of Vitamin D Deficiency Rickets Caused by Feeding Problems with Autism Spectrum Disorder
Author
Akina Matsuoka Katsuyuki Matsui Jun Matsui Noriko Nishikura Seiichiro Yoshioka Yoshihiro Maruo Tomoyuki Takano and Yoshihiro Takeuchi
Department of Pediatrics, Shiga University of Medical Science
Abstract
The incidence of pediatric vitamin D deficiency-related rickets has been increasing. We present a case of vitamin D deficiency-related rickets caused by feeding problems in a patient with autism spectrum disorder. A 3-year-old boy was hospitalized with a seizure disorder and hypocalcemia. The electrocardiogram revealed a prolonged QT interval, and X-ray examination of the wrist and knee joints showed cupping, fraying, and flaring changes. Serum testing showed a high level of parathyroid hormone and very low level of 25-hydroxyvitamin D, suggesting vitamin D deficiency rickets. He was diagnosed with autism spectrum disorder because of social communication deficits and speech delay. He also manifested feeding problems with deviated food habits and resultant vitamin D deficiency. He was treated with vitamin D and calcium. His parents consulted psychotherapy for his social communication deficits, and several intervention programs were designed by local community services.
We suggest that an inadequate educational environment and insufficient medical or community intervention may be risk factors for nutritional disorders in patients with autism spectrum disorder.




Case Report
Title
A Case of Severe Combined Immunodeficiency with Vaccine-acquired Rotavirus
Author
Takahiro Tomoda1) Noriko Mitsuiki1) Tsubasa Okano1) Mari Tanaka-Kubota1) Satoshi Miyamoto1) Shunsuke Kimura1)2) Masatoshi Takagi3) Kousuke Imai3) Michiko Kajiwara4) Hirokazu Kanegane1) and Tomohiro Morio1)
1)Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
2)Department of Pediatrics, St Luke's International Hospital
3)Department of Community Pediatrics, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
4)Department of Transfusion, Tokyo Medical and Dental University
Abstract
We describe a 9-month-old boy with severe combined immunodeficiency (SCID), in whom rotavirus antigen was persistently detected in stool. The patient developed mild diarrhea alone, and the rotavirus was not detected following cord blood transplantation. The rotavirus detected in the patient might have originated from oral live attenuated rotavirus vaccine which was administered before a diagnosis of SCID. We performed rotavirus gene analysis of the samples from the patient and vaccine, and these sequences were quite similar, and were different from the wild-type. This is the first report in Japan that the rotavirus from a SCID patient was identified to be derived from oral live attenuated vaccine. It was reported that SCID patients might develop severe gastroenteritis associated with vaccine-acquired rotavirus. SCID patients may present with overt infection caused by live attenuated vaccine, but it may be difficult to diagnose asymptomatic SCID patients before 2 months of age. Neonatal mass screening with the Guthrie card is useful for the early diagnosis of SCID patient to avoid live attenuated vaccine administration, and it should be introduced in Japan as well as in United States.




Case Report
Title
Zinc Deficiency in Low Zinc Breast Milk Related to Maternal ZnT2 Gene Mutation
Author
Takafumi Obara1) Nagisa Komatsu1) Naoya Itsumura2) Taiho Kambe2) Yuichiro Muto1) Takahiro Nishihara1) Katsuki Hirai1) and Masahiro Migita1)
1)Department of Pediatrics, Japanese Red Cross Kumamoto Hospital
2)Department of Applied Molecular Biology Division of Integrated Life Science, Graduate School of Biostudies Kyoto University
Abstract
Zinc deficiency in infants affects various symptoms including dermatitis, alopecia and growth retardation. Low zinc breast milk is one of reasons for zinc deficiency in infants, which is named transient neonatal zinc deficiency (TNZD). Recent studies have revealed that Zn transporter 2 (ZnT2) plays an essential role in the secretion of zinc into breast milk and its mutations are responsible for TNZD pathogenesis. Here, we report the case of an infant zinc deficiency caused by low zinc breast milk, which was attributed to a novel maternal ZnT2 gene mutation. Preterm female baby (gestational week 32 weeks; birth weight 1,772 g) had been only breast-fed. Three months later, she displayed atopic-like dermatitis on her face and limbs. First visited at the age of 5 months, she also had alopecia and growth retardation (BH: 60.0 cm (-1.95 SD), BW: 6.075 kg). Her serum low zinc levels (15 μg/dl) and the deviation between mother's serum zinc levels and breast milk (81 μg/dl vs. 31 μg/dl) were suspected to be TNZD. We identified the c.652C>T heterozygous nonsense mutation in maternal SLC30A2/ZnT2 gene, which suggested that haploinsufficiency of SLC30A2/ZnT2 gene led to TNZD in this case. Consistent with this, her symptoms improved immediately after prescription of an orally medicine containing an L-carnosine zinc complex. Our results indicate that there might be more potential patients of TNZD, and therefore pediatricians should be more carefully to examine infants suffering from dermatitis, alopecia and growth retardation.




Case Report
Title
A Boy of Polyarteritis Nodosa with Coronary Artery Lesion Who Attained Remission with Plasma Exchange and High Dose Glucocorticoid Therapy
Author
Hiroshi Kubo1) Taeko Saito1) Junko Dou1) Kazutaka Ouchi1) Kazuyuki Ikeda2) Kenji Hamaoka2) Shinji Akioka1) and Hajime Hosoi1)
1)Department of Pediatrics, Kyoto Prefectural University of Medicine
2)Department of Pediatric Cardiology and Nephrology, Kyoto Prefectural University of Medicine
Abstract
A 15-year-old boy was admitted to our hospital due to persistent fever, systemic pain, erythema-like rush and diarrhea. Coronary artery lesions (CAL) also developed just after the admission, which was resistant to intravenous immunoglobulin therapy (IVIG). In the tentative diagnosis of systemic vasculitis rather than Kawasaki disease, plasmapheresis led to clinical remission and clearance of CAL, but did not achieve normalization of serological biomarkers. One month later, fever reappeared with recurrence of CAL, which was resistant to anti-inflammatory drugs, IVIG, cyclosporine A and infliximab same as before. Multiple intervention with repeated plasmapheresis, high-dose glucocorticoid pulse therapy and subsequent intravenous cyclophosphamide infusions successfully induced regression of CAL with complete remission both clinically and serologically. He was finally diagnosed with polyarteritis nodosa on the basis of imaging findings, recurrent CAL, non-traumatic hemorrhage in renal cyst and inflammatory changes in muscles of lower extremities. He is now keeping complete remission for 2 years without any sequela under mycophenolate mofetil monotherapy after termination of short-term glucocorticoid treatment. In the early stage of primary vasculitis in childhood, it is difficult to obtain a definitive diagnosis except for the typical case of Kawasaki disease. However, mortal organ involvement such as CAL might be recognized even at the onset, which definitely requires rapid managements with distinct efficacy and high-level safety in order to avoid sequelae. Our case suggests that even under the situation without the definite diagnosis, appropriate interventions should be done under consideration of pathophysiological conditions and processes especially focused on organ-threatening lesions along with the safety of operations.




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