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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.122, No.11, November 2018


Original Article
1. Issues with Childcare for Infants with Congenital Heart Disease and Family Problems
2. A Study on Appropriate Vitamin D Supplementation in Vitamin D-deficient Breastfed Infants
3. Local Adjusted Neonatal Palivizumab Inoculation Schedule
4. Air Medical Transport for Paediatric Medical Emergencies
Case Report
1. A Case of Primary Central Nervous System Lymphoma, Indistinguishable from Acute Disseminated Encephalomyelitis
2. A Case of Fulminant Type 1 Diabetes with Hyperglycemic Hyperosmolar State at Onset
3. A Case of Cat-scratch Disease with Relative Bradycardia
4. A 4-year-old Girl Pathologically Diagnosed as Eosinophilic Bronchiolitis
5. IgA Vasuculitis in a 4-year-old Boy with Syndrome of Inappropriate Secretion of Antidiuretic Hormone and Protein-losing Enteropathy


Original Article
Title
Issues with Childcare for Infants with Congenital Heart Disease and Family Problems
Author
Mayuko Shohi Masahiro Kamada Naomi Nakagawa Yukiko Ishiguchi Yuji Morito Yoshimi Matsumoto and Kengo Okamoto
Department of Pediatric Cardiology, Hiroshima City Hiroshima Citizen's Hospital
Abstract
Background: Children who have both congenital heart disease (CHD) and family problems may experience delayed hospital discharge regardless of their stable condition. We investigated the factors impairing childcare at home and issues with a social childcare system.
Methods: Case series: we conducted a retrospective review of 6 CHD patients requiring longer hospitalization than expected owing to poor childcare at home. Questionnaire study: we mailed a questionnaire to all seven infant homes in the Chugoku region and surveyed the circumstances of CHD patient admission and their problems regarding childcare.
Results: Two patients were admitted to infant homes, one was adopted, two were discharged, and one died in the hospital. The clinical conditions of 3 patients receiving social support were stable. Maternal mental disorders (83%) and absence of familial childcare support (83%) were identified as major problems. The questionnaire response was 100%. Six infant homes (85.7%) admitted CHD infants if they had no clinical symptoms (71.4%), had successful surgeries (57.1%), and did not need domiciliary oxygen therapy (42.9%).
Discussion: Maternal poor health and the absence of familial support were found to impair childcare at home. Moreover, CHD patients with residual symptoms experienced difficulties in receiving social childcare support. Therefore, enhanced cooperation between infant homes and hospitals and increased social support for children requiring medical care is critical.




Original Article
Title
A Study on Appropriate Vitamin D Supplementation in Vitamin D-deficient Breastfed Infants
Author
Kazuhiko Tomimoto
Tomimoto Children's Clinic
Abstract
[Background] Vitamin D deficiency is a concern in breastfed Japanese infants, however, the optimum supplementation has yet to be determined.
[Methods] In a randomized controlled trial, 91 infants who were breastfed and with a 25OHD level below 20 ng/mL received an additional 160 IU or 400 IU of vitamin D daily for 4 weeks. After supplementation, we examined the number of subjects with a 25OHD level below 20 ng/mL, and the changes in levels of 25OHD, PTH, ALP, Ca, and P.
[Results] After supplementation, a 25OHD level below 20 ng/mL was observed in 11 of 45 infants receiving 160 IU of vitamin D daily and 1 of 46 infants receiving 400 IU of vitamin D daily (P=0.0017). The 25OHD level was 26 (IQR: 20-30) ng/mL in infants receiving 160 IU of vitamin D daily, and 29 (IQR: 24-32) ng/mL in those receiving 400 IU (P=0.012). The 25OHD level increased in a dose-dependent manner, but the levels of PTH, ALP, Ca, and P did not differ significantly between the 2 groups.
[Discussion] Some of the infants receiving 160 IU of vitamin D daily remained in a vitamin D-deficient condition, still leaving risk of rickets. The supplementation dose of vitamin D should be 400 IU daily.
[Conclusion] The infants who are almost exclusively breastfed, and also live in the regions at high latitudes or with few hours of daylight, should receive an additional 400 IU of vitamin D daily.




Original Article
Title
Local Adjusted Neonatal Palivizumab Inoculation Schedule
Author
Tomoko Makiya1) Tomohide Yoshida2) Chiaki Ohba3) Tomoko Uehara4) Kazuhiko Nakasone5) Masayuki Isa6) Yoriko Kisato1) Ayako Izumi1) and Moriyasu Kohama1)
1)Division of Neonatology, Okinawa Chubu Hospital
2)Division of Neonatology, University of Ryukyus Hospital
3)Division of Neonatology, Okinawa Nanbu Medical Center & Children's Medical Center
4)Department of Pediatrics, Naha City Hospital
5)Department of Pediatrics, Okinawa Red Cross Hospital
6)Department of Pediatrics, Okinawa Hokubu Hospital
Abstract
RSV infection occurs throughout the year in Okinawa. In 2008, the Okinawa Neonatal Study Group (ONSG) developed its first guidelines for Palivizumab inoculation. Infants were divided into three risk groups based on gestational age (GA); Group A: 22-28 weeks, Group B: 29-32 weeks, and Group C: 33-35 weeks. Starting at the time of NICU discharge, infants in Group A received monthly Palivizumab inoculations until they were 12 months old. Infants in both Groups B and C received monthly post-discharge inoculations until they were 6 months of age.
In 2013 a multicenter study was carried out to evaluate the effectiveness of the inoculation schedule. The study looked at re-admission rates of ex-premature infants with RSV infection based on GA. A total of 927 ex-premature infants were followed.
Sub-analysis showed that infants in Group B who had received ≤ 3 doses of Palivizumab had a higher admission rate than all other groups. Therefore ONSG increased this group's number of post-discharge doses of Palivizumab to at least 4, even for those who had already passed age 6 months. Additional evaluation is recommended to further optimize Palivizumab inoculation guidelines.




Original Article
Title
Air Medical Transport for Paediatric Medical Emergencies
Author
Takuro Iwasaki1) Naohito Arii1) Yosuke Baba1) Minoru Arii1) Natsuki Okawa2) Masato Kantake1)2) and Toshiaki Shimizu3)
1)Department of Pediatrics, Juntendo University Shizuoka Hospital
2)Department of Neonatology, Juntendo University Shizuoka Hospital
3)Department of Pediatrics, Juntendo University Faculty of Medicine
Abstract
We assessed the feasibility of air medical transport for paediatric medical emergencies in the eastern part of Shizuoka Prefecture.
Data were collected from the transcripts of air medical transport calls from April 2004 to March 2016. With the exclusion of newborns, cases of patients aged < 15 years were included and categorised by sex, age, primary disease type, dispatch request, requester and transport destination. A comparison between data from the first and last six years was drawn.
Of the 604 selected cases, 379 were male and 225 were female. Categorising by primary disease type revealed 283 endogenous cases and 321 exogenous cases. Categorising by dispatch request revealed 314 on-site dispatches and 290 inter-hospital transfers.
In comparison to the first six years, in the last six years, the number of inter-hospital transfers due to endogenous diseases decreased, while the number of on-site dispatches due to exogenous diseases increased. The dispatch requests were made from all regions within this district, with our hospital and a children's specialised hospital as the predominant transfer destinations.
Air medical transport is suitable for patient transfer in the mountainous terrain of Shizuoka Prefecture. It is important to understand the underlying advantages and disadvantages of air medical transport to allow for its effective use in paediatric medical emergencies.




Case Report
Title
A Case of Primary Central Nervous System Lymphoma, Indistinguishable from Acute Disseminated Encephalomyelitis
Author
Nozomi Tanabe Yuichiro Muto Yusuke Miyashita Shuichi Yanabe Takahiro Nishihara Nagisa Komatsu Katsuki Hirai and Masahiro Migita
Department of Pediatrics, Japanese Red Cross Kumamoto Hospital
Abstract
Pediatric acute disseminated encephalomyelitis (ADEM) is a disease with diffuse white matter changes on magnetic resonance imaging (MRI) that are similar to those of central nervous system (CNS) malignant neoplasms. Herein, we report a pediatric case of primary CNS lymphoma (PCNSL) with diffuse white matter changes on brain MRI.
A 1-year-old female presented with acute consciousness disturbance and ptosis. Her cerebrospinal fluid cell count was elevated, and brain MRI revealed diffuse white matter changes. Accordingly, we diagnosed ADEM and prescribed steroid pulse therapy. Although her cognitive status initially improved, it deteriorated again. MRI revealed lesion expansion; despite repeated steroid pulses, her condition worsened. We performed brain biopsy and diagnosed PCNSL. We found a large number of Epstein-Barr virus (EBV) positive cells within the tumor; thus, the diagnosis was revised as EBV-related malignant lymphoma, probably associated with immunodeficiency. Although whole exome analysis did not identify any particular immunodeficiency disease, we suspected an unknown immunodeficiency genotype. Remarkably, after chemotherapy with high-dose methotrexate, the white matter changes on MRI disappeared. However, severe psychomotor deterioration persisted due to severe brain atrophy.
Although PCNSL is a very rare disease, it should be considered as a cause of diffuse white matter changes on MRI.




Case Report
Title
A Case of Fulminant Type 1 Diabetes with Hyperglycemic Hyperosmolar State at Onset
Author
Kumi Chougahara1) Kazuhiko Jinno1) Risa Morita1) Atsuko Okuda1) Hiroaki Ono1) Hiroshi Fujii2) and Toshiyuki Ohta2)
1)Department of Pediatrics, Hiroshima Prefectural Hospital
2)Department of Pediatric Nephrology, Hiroshima Prefectural Hospital
Abstract
A female patient, aged 13 years and 8 months, had consumed 4 to 5 liters of soft drinks each day for three days before hospitalization because she was thirsty. Her level of consciousness declined, and she was presented to, and hospitalized in an emergency hospital with blood glucose levels of 1,090 mg/dL, glycated hemoglobin levels of 6.2%, plasma osmolality of 341 mOsm/L, pH of 7.081, bicarbonate levels of 7.5 mmol/L, urinary ketone body level of 2+, urinary C peptide levels of 1.5 μg/day. Based on these values, she was diagnosed with fulminant type 1 diabetes mellitus with diabetic ketoacidosis (DKA) mixed with hyperglycemic hyperosmolarity. In addition to disturbance of consciousness, acute renal failure accompanied with dehydration, rhabdomyolysis, and liver dysfunction; however, these were restored following treatment.
Cases of mixed hyperglycemic hyperosmolar syndrome (HHS) and DKA may be encountered, in which HHS and DKA concur at the time of the onset of type 1 diabetes; however, there have been few reports of such cases in children. One of the causes of mixed HHS and DKA is presumed to be the consumption of sugary beverages, such as soft drinks, suggesting a similar possibility in this case as well. Mixed HHS and DKA is accompanied by severe dehydration in affected patients. Because complications tend to develop, proper treatment to improve blood glucose and electrolyte levels should be ensured more carefully than in patients with DKA.




Case Report
Title
A Case of Cat-scratch Disease with Relative Bradycardia
Author
Junya Tsukamoto Hiroshi Katsumori Tomoki Kawahara and Ryo Kozawa
Department of Pediatrics, Kawakita General Hospital
Abstract
A 13-year-old boy presented with fever on disease day 1 and was prescribed cefditoren pivoxil and tosufloxacin by a local doctor but a remittent fever of 39-40℃ persisted. On disease day 7, he presented to our hospital's emergency room complaining of abdominal pain, and diarrhea. He was admitted, and bacterial enterocolitis was suspected. On admission, relative bradycardia (40.5℃ 85 beats/min) and slight swelling of the inguinal lymph nodes were observed. He had had contact with a patient with Salmonella enteritis; therefore, bacteremia caused by Salmonella enterica was suspected. Intravenous cefotaxime was administered, which led to improvement of the symptoms; however, the remittent fever persisted. The blood culture was negative, and stool culture showed only normal flora. Since he had a history of caring for a cat, Bartonella antibodies were tested to differentiate cat-scratch disease. His medication was then switched to intravenous minocycline on disease day 11. His fever was resolved on day 15, and he was discharged. The test showed elevated serum levels of Bartonella antibodies, which confirmed the diagnosis of cat-scratch disease.
Based on our literature review, there are no reports that indicated an association between cat-scratch disease and relative bradycardia. However, upon examining previous reports for temperature and pulse rate findings, relative bradycardia was noted in 7 of 16 reports, including the present case. This indicates that relative bradycardia may also be observed in cat-scratch disease.




Case Report
Title
A 4-year-old Girl Pathologically Diagnosed as Eosinophilic Bronchiolitis
Author
Takafumi Takase1) Yoshitaka Miyoshi1) Yoshitaka Nakamura2) and Ryugo Hiramoto1)
1)Department of Pediatrics, Matsudo City General Hospital Children's Medical Centre
2)Department of Pediatrics, Tako Central Hospital
Abstract
Eosinophilic bronchiolitis is a new disease concept that was first reported in an adult in Japan in 2001, and there have been some reports of other adult cases since then. There are, however, no reports of child cases as far as we know. We here report a 4-year-old girl pathologically diagnosed as having eosinophilic bronchiolitis. The patient was diagnosed with bronchial asthma at the age of 18 months and has been continuously treated for bronchial asthma. Because inhaled steroids were not effective enough and a blood test revealed a great increase in eosinophils, we diagnosed the patient with intractable asthma. Eosinophilic bronchiolitis was suspected because only oral administration of prednisolone efficiently improved the symptoms and decreased the blood eosinophilic count, and also a chest CT scan showed centrilobular nodules. Bronchoalveolar lavage demonstrated marked eosinophilia and a video-assisted thoracoscopic lung biopsy showed bronchiolitis with eosinophilic infiltration. Eosinophilic bronchiolitis was finally diagnosed based on these findings. The possibility of eosinophilic bronchiolitis should be considered, and a chest CT scan and bronchoalveolar lavage should be planned even in children when bronchial asthma accompanied by marked eosinophilia responds only to oral administration of prednisolone.




Case Report
Title
IgA Vasuculitis in a 4-year-old Boy with Syndrome of Inappropriate Secretion of Antidiuretic Hormone and Protein-losing Enteropathy
Author
Yuji Fujita1) Daisuke Sawada1) Mai Masuda2) Miyuki Hayashi1) Nobuyuki Takada1) Kaori Kinoshita1) Takayasu Arima1) Chieko Matsumura2) and Shinichi Suwabe1)
1)Department of Pediatrics, Kimitsu Chuo Hospital
2)Department of Pediatrics, National Hospital Organization Chiba-East Hospital
Abstract
IgA vasculitis (IgAV) is the most common type of systemic vasculitis in children. Here, we report a case of IgAV in a 4-year-old boy with protein-losing enteropathy and syndrome of inappropriate secretion of antidiuretic hormone (SIADH).
The boy presented with vomiting and abdominal pain on the first day. On the sixth day he had hyponatremia, and was diagnosed with SIADH due to the level of urine sodium and antidiuretic hormone. On the eighth day, IgAV was suspected owing to high levels of D-dimer, and he was administered with prednisolone. On the ninth day, he was diagnosed with protein-losing enteropathy due to hypoalbuminemia, watery stool, and ascites. On the 10th day, he was given a diagnosis of IgAV due to purpura on the extremities. He looked pale, the abdominal pain exacerbated, and blood examination revealed a low level of factor XIII activity; he was given factor XIII. On the same night, his abdominal pain, hyponatremia and hypoalbuminemia improved.
Hyponatremia is often observed in Kawasaki disease (KD). Some reports revealed that hyponatremia in KD may be associated with SIADH. However, a case of IgAV with SIADH from outside Japan has not been reported, and only one adult patient was reported from our country. When hyponatremia is recognized in IgAV, it should be suspected to be SIADH.




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