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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.122, No.8, August 2018


Review
1. Potential Benefits and Drawbacks of High Flow Nasal Flow Therapy in Practice in Pediatrics
Original Article
1. Relationship between Renal Length and Renal Function Prognosis by Ultrasonic Tomography in Single Kidneys
2. Impact of Pediatric Intensive Care Unit on the Centralization and Mortality of Critically Ill and Injured Children
3. Fasting before Sedation Improves Magnetic Resonance Imaging Success Rates in Young Children
Case Report
1. Congenital Chylothorax Caused by Mediastinal Neuroblastoma
2. Three Cases of Scimitar Syndrome with Various Clinical Manifestations
3. A Case of Limbic Encephalitis Initially Presenting with Urinary Retention
4. Successful Management of an Infant with Kaposiform Hemangioendothelioma Complicated by Spinal Canal Invasion Using Sirolimus
5. Combination Therapy Using Prednisolone, Cyclosporine A, and Mycophenolate Mofetil in a Patient with Refractory Grade VI Henoch-Schönlein Purpura Nephritis
6. A case of Pediatric Clostridium Difficile Infection after Exposure to Oral Antibiotics for Several Days
7. A 2-year-old Girl with Scurvy, Leg Pain, and Gait Disturbance Caused by an Unbalanced Diet


Review
Title
Potential Benefits and Drawbacks of High Flow Nasal Flow Therapy in Practice in Pediatrics
Author
Atsushi Kawaguchi1) and Muneyuki Takeuchi2)
1)Department of Pediatrics, Pediatric Critical Care Medicine, University of Alberta
2)Division of Pediatric Critical Care, Osaka Women's and Children's Hospital
Abstract
High Flow Nasal Cannula (HFNC) therapy has been recognized as an important option for non-invasive respiratory support of critically ill children with respiratory distress/failure. The use of HFNC for neonates and infants has been rigorously studied; conversely, the evidence supporting HFNC therapy for older children is limited. Having a better understanding of physiological effects and potential benefits and drawbacks in the practice of pediatric HFNC, and in particular, for critically ill children, will allow us to provide a better and safer practice, but also to devise future research. This article summarizes the current knowledge and evidence with regard to the potential positive and negative aspects of HFNC in contemporary pediatric practice.




Original Article
Title
Relationship between Renal Length and Renal Function Prognosis by Ultrasonic Tomography in Single Kidneys
Author
Tomoki Goto1) Yoshimitsu Gotoh2) and Osamu Uemura3)
1)Department of Pediatrics, Yokkaichi Municipal Hospital
2)Department of Pediatric Nephrology, Japanese Red Cross Nagoya Daini Hospital
3)Clinical Medicine, Japanese Red Cross Toyota College of Nursing
Abstract
OBJECTIVE: Renal length in Japanese healthy children has been examined, but that in pediatric single kidneys has not. The relationship between renal length measured by ultrasound examination and renal function was examined.
METHODS: We examined 45 pediatric single kidneys undergoing follow-up at 2 hospitals and retrospectively investigated their medical records.
RESULTS: Renal length correlated well with age and height (correlation coefficient: age 0.87, height 0.91). The regression equation of renal length estimated from body height was 0.0529×height (cm)+2.67. Renal function was evaluated by dividing kidneys into two groups large (N) and small (S) with renal length presumed from body height. The median estimated glomerular filtration rate (eGFR) was significantly lower in S than N group, 83.6 mL/min/1.73 m2 vs 96.0 mL/min/1.73 m2, respectively (p<0.01). On the final observation, we grouped kidneys by CKD stage 2 and CKD stage 1. In many cases of CKD stage 2 group, the renal length was shorter than the presumed renal length.
CONCLUSION: Because single kidneys with shorter renal length than assumed had low eGFR, it was suggested that renal function could be indirectly evaluated. On follow-up of single kidneys, it is considered useful to measure renal length by ultrasonography to avoid invasive examination.




Original Article
Title
Impact of Pediatric Intensive Care Unit on the Centralization and Mortality of Critically Ill and Injured Children
Author
Ryuta Itakura1) Yoshio Sakurai1) Ai Miyamoto1) Shingo Kobayashi1) Kohei Osada1) Kenji Sugamoto1) Tomoko Adachi1) Teruichiro Kitaoka1) Hirokazu Sakai1)2) and Masanori Tamura1)2)
1)Division of Pediatric Critical Care Medicine, Saitama Medical Center, Saitama Medical University
2)Department of Pediatrics, Saitama Medical Center, Saitama Medical University
Abstract
Background: The pediatric intensive care unit (PICU) of our institute, established in March 2016, was the ninth pediatric tertiary institute in Japan. Before that, a majority of critically ill and injured pediatric patients in Saitama Prefecture were referred to non-tertiary pediatric units.
Purpose: This study aimed to assess the impact of establishing the PICU on the centralization and mortality of critically ill and injured children in Saitama Prefecture.
Methods: We retrospectively reviewed the charts of patients who were admitted to our PICU between March 2016 and February 2017.
Results: During the study period, 402 patients were admitted to our PICU; of these, 76.7% of patients required admission from out-of-hospital and 87.6% required emergency admission. In addition, 151 patients were transferred from other hospitals from all regions of Saitama Prefecture. Of these, 97 patients (64.2%) were transported by our medical transport team. In this study, the mean predictive mortality rate per Pediatric Mortality Index 2 (PIM2) was 5.8%, whereas the actual mortality rate was 2.6%.
Conclusion: Establishment of an efficient transport system for critically ill and injured children, besides centralizing these patients in a PICU, has been shown to be beneficial in Saitama Prefecture.




Original Article
Title
Fasting before Sedation Improves Magnetic Resonance Imaging Success Rates in Young Children
Author
Takuya Masuda1) Mari Saito1) Yutaka Kikuchi1) Masanori Kurosaki1)2) Saori Fukui1) Hironori Shimozawa1)2) and Masaru Hoshina1)
1)Department of Pediatrics, Haga Red Cross Hospital
2)Department of Pediatrics, Jichi Medical University
Abstract
Background: The Japan Pediatric Society and two other academic societies released a joint statement to recommend fasting before sedation (2-4-6 rule) for young children. However, more than half of hospitals had not followed the rule, since data on how this joint statement affected MRI success rates are lacking. In this study, we analyzed MRI success rates before and after the introduction of the 2-4-6 rule. Methods: All children under 6 years old who underwent MRI under sedation between April 2016 to November 2017 were enrolled in this study. Children who underwent MRI before February 2017 were enrolled retrospectively in the pre 2-4-6 rule group and those who underwent MRI in or after February 2017 were enrolled in the post 2-4-6 rule group. Results: Fifty-nine patients comprised the pre 2-4-6 rule group, and 50 patients comprised the post 2-4-6 rule group. The MRI success rate was significantly higher in the post 2-4-6 rules groups (83.0% vs 98.0%, P=0.0098) with improved success rate by 1.4 times in children 3 years of age. There were no differences between groups in ratios of gender, outpatients, additional use of sedation, and frequency of adverse events. Conclusions: The 2-4-6 rule improved the MRI success rate under sedation in young children.




Case Report
Title
Congenital Chylothorax Caused by Mediastinal Neuroblastoma
Author
Sachiko Onodera1) Yu Katata1) Masae Kobayashi1) Yuichiro Miura1) Toshihiko Uchida1) Tatsuya Watanabe1) Junko Saito1) Nobu Suzuki2) Atsushi Sato2) and Masue Imaizumi2)
1)Department of Neonatology, Miyagi Children's Hospital
2)Department of Hematology and Oncology, Miyagi Children's Hospital
Abstract
Congenital chylothorax of unknown cause sometimes occurs. Moreover, congenital neuroblastoma can develop at 30-36 weeks of gestation sometimes leading to hydramnion and fetal hydrops.
We report a case of congenital chylothorax that was suspected to be associated with a mediastinal neuroblastoma. At 31 weeks of gestational age, pleural effusion was detected and she was born at 33 weeks by cesarean section because of fetal hydrops. As total parental nutrition was not sufficient, prednisolone and octreotide were administered. The pleural effusion stopped at 15 days. Chest radiography revealed dextroversion of the mediastinum and a shadow suspected to be a tumor in the left lung field. Computerized tomography (CT) scanning of the chest at 28 days, revealed a 3 cm tumor and calcification in the left posterior mediastinum. MIBG scintigraphy revealed accumulation of 123I-MIBG in the tumor. Her serous NSE and VMA/HVA values in urine samples were elevated. On the basis of these findings, the tumor was diagnosed as neuroblastoma, and no metastasis was indicated. Because of the lack of amplification of serous MYCN gene, the neuroblastoma was considered to be of the low-risk type. The patient received no treatment.
Analyzing the location and size of the tumor, and the course of chylothorax, we concluded that the chylothorax was caused by the mediastinal neuroblastoma. Thus, in patients with congenital chylothorax, an intrapleural tumor must be ruled out.




Case Report
Title
Three Cases of Scimitar Syndrome with Various Clinical Manifestations
Author
Hiroyuki Nagao Toshikatsu Tanaka Naoya Kamei Michio Mathuoka Yoshiharu Ogawa Kenta Tominaga and Sachiko Kido
Department of Cardiology, Hyogo Prefectural Kobe Children's Hospital
Abstract
Scimitar syndrome is a rare disease associated with abnormal pulmonary venous drainage into the inferior vena cava. Right lung sequestration and pulmonary hypertension are common complications. Case 1 was of a 4-year-old boy incidentally diagnosed with Scimitar syndrome. At age 16 years, he underwent cardiac catheterization but no surgical indications were identified.
Case 2 was a 7-month-old girl. After colostomy closure, she developed acute respiratory failure, and was diagnosed with Scimitar syndrome by echocardiography. Cardiac catheterization revealed stenosis at the junction of the inferior vena cava and partial anomalous pulmonary venous return. Surgical repair was performed at the age of 10 months, but the right pulmonary vein was occluded. Release of the occlusion was performed, but the vein again became totally occluded.
Case 3 was a 6-month-old girl. Scimitar syndrome was suspected on fetal echocardiography. Cardiac catheterization showed a Scimitar vein and the right pulmonary artery was hypoplastic. Major aortopulmonary collateral arteries from the abdominal aorta provided right lung perfusion, and sequestration was diagnosed. We only performed closure for artery feeding the pulmonary sequestration using coil embolization. The patient was followed up in the outpatient clinic.
Scimitar syndrome has various complications and degrees of severity; thus, individual hemodynamics should be considered in determining the optimal timing and method of intervention.




Case Report
Title
A Case of Limbic Encephalitis Initially Presenting with Urinary Retention
Author
Takuji Nakamura1)2) Muneaki Matsuo2) and Masafumi Zaitsu1)2)
1)Department of Pediatrics, National Hospital Organization Ureshino Medical Center
2)Department of Pediatrics, Faculty of Medicine, Saga University
Abstract
We describe a 13-year-old girl with limbic encephalitis who was admitted based on an initial presentation of urinary retention. She had fever that had persisted for 8 days before admission. Thereafter, she gradually developed delirium with emotional changes, impulsive behavior, unresponsiveness, and sinus bradycardia. Brain MRI showed hyperintense lesions in the right temporal and parietal lobes and the insular cortex in the acute phase. These findings fulfilled the diagnostic criteria for limbic encephalitis. Coxsackie virus B5 was isolated from her stool. She was treated with pulsed intravenous methylprednisolone and intravenous immunoglobulin, which improved her level of consciousness and the delirious behavior. Urinary retention persisted for 22 days after admission. Limbic encephalitis in children initially presenting with urinary retention has not been studied in detail. Limbic encephalitis should be considered as a possible cause of urinary retention.




Case Report
Title
Successful Management of an Infant with Kaposiform Hemangioendothelioma Complicated by Spinal Canal Invasion Using Sirolimus
Author
Chiaki Takemori Koji Kawaguchi Ikuko Takahashi Taemi Ogura Yasuo Horikoshi and Kenichiro Watanabe
Department of Hematology and Oncology, Shizuoka Children's Hospital
Abstract
Corticosteroids in combination with vincristine are considered a first-line therapy for Kaposiform hemagioendothelioma (KHE) complicated by Kasabach-Merritt phenomenon. However, many cases are poorly responsive or refractory to these therapies. Recently, sirolimus, a mammalian target of Rapamycin inhibitor (mTOR inhibitor), was found to be effective for the treatment of KHE patients. We encountered a case of KHE with associated KMP, and spinal canal invasion that was successfully managed by sirolimus therapy.
A 25-day-old baby girl presented with hemangioma and subcutaneous hemorrhage. Blood examination revealed thrombocytopenia, significantly elevated D-dimer level, and decreased fibrinogen level. Magnetic resonance imaging showed a large left-sided retroperitoneal mass, which was consistent with a diagnosis of KHE. Therapy was started with prednisolone, propranolol, and vincristine; however, the tumor progressed, and it was impossible to wean platelet transfusion. Moreover, there was a high risk of spinal cord paralysis due to tumor progression. A tumor biopsy performed when the patient was 93 days old confirmed the diagnosis of KHE, and sirolimus therapy was initiated. Eight days after, it was possible to wean the patient from platelet transfusion. Over the course of six months, the size of the tumor decreased significantly. Thus, sirolimus has the potential to suppress tumor growth and block the development of neurological complications caused by spinal invasion of the tumor. Therefore, we recommend sirolimus as a first-line therapy for KHE patients with invasive lesions that may cause severe complications.




Case Report
Title
Combination Therapy Using Prednisolone, Cyclosporine A, and Mycophenolate Mofetil in a Patient with Refractory Grade VI Henoch-Schönlein Purpura Nephritis
Author
Shunsuke Noda1) and Daisuke Matsuoka2)
1)Department of Pediatrics, Nagano Red Cross Hospital
2)Department of Pediatrics, Shinshu University
Abstract
An 8-year-old boy presented with gradeVI Henoch-Schönlein purpura nephritis. Despite prednisolone (PSL), mizoribin, dipyridamole and warfarin administration, he did not show improvement. Therefore, we initiated treatment with cyclosporine A (CYA). He caught a cold, and his urinary protein increased. We initiated treatment with mycophenolate mofetil (MMF) in combination with CYA. Proteinuria decreased with CYA, MMF, and PSL combination therapy, and the patient's nephritis improved. Tonsillectomy was performed on day 145 to prevent the recurrence of nephritis caused by infection.
A renal biopsy showed disease improvement from grade VI to grade II.
The efficacy of treatment with CYA and MMF has been reported in severe cases, but to our knowledge, no large-scale clinical trial has been performed to date. Plasma exchange therapy has also been attempted, but it is only effective at an early stage of the onset and in case of high invasion. Since there was less degree of invasion in this case, a therapeutic effect was successfully achieved.




Case Report
Title
A case of Pediatric Clostridium Difficile Infection after Exposure to Oral Antibiotics for Several Days
Author
Toshifumi Yodoshi1) Itaru Iwama2) Yoshiki Kusama3) Mizue Kishida1) Kei Matayoshi1) Taisuke Tsuji1) Saori Kinjo1) and Moriyasu Kohama1)
1)Department of Pediatrics, Okinawa Chubu Hospital
2)Division of Gastroenterology and Hepatology, Saitama Children's Medical Center
3)AMR Clinical Reference Center, National Center for Global Health and Medicine Hospital
Abstract
Clostridium difficile infection (CDI) is one of the most common antimicrobial-associated diarrheas among adults in Japan. However, there are few pediatric reports of CDI in outpatient settings. We report a case of CDI that developed after taking oral antibiotics for several days. A 5-year-girl was referred to our hospital with fever, diarrhea and abdominal pain. She had been taking 3rd generation cephalosporin and fosfomycin orally for several days after being given a diagnosis of colitis at nearby two clinics. Abdominal ultrasonography demonstrated a thickened bowel wall, extending from the rectum to the descending colon. She was diagnosed with CDI after a positive test result was obtained for Clostridium difficile toxins A/B. A 10-day course of metronidazole resulted in clinical improvement.
As is the case in adults, we reiterate that it is important not to prescribe antibiotics for mild enteritis in children in order to avoid facilitate the potential onset of CDI. It is important to obtain a complete history of all antimicrobials taken within the past 60 days in patients with a history of enteritis. Ultrasound detection of a thickened wall in the left semicolon may serve as a good supplemental diagnostic test for CDI.




Case Report
Title
A 2-year-old Girl with Scurvy, Leg Pain, and Gait Disturbance Caused by an Unbalanced Diet
Author
Yotaro Hanami Jyunya Abe Atsushi Arai Noriko Maida Hideto Ogata Sumie Yamashita and Daisuke Hata
Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute
Abstract
Scurvy is a rare disease caused by vitamin C deficiency. Here, we report a 2-year-old infant with scurvy resulting from an extremely unbalanced diet. She also suffered from leg pain and gait disturbance. Magnetic resonance imaging showed multifocal lesions in her femurs and tibia; however, positron emission tomography-computed tomography, bone scintigraphy, and bone marrow examination did not detect malignant disease. She had a history of an extremely unbalanced diet lacking all vegetables and fruits, and she had gingival bleeding and hematuria. Additional blood examination detected vitamin C deficiency. Upon oral vitamin C supplementation, her leg pain, mobility, and gingivitis rapidly improved. Based on these findings, we diagnosed scurvy. Unfortunately, the clinical manifestations of scurvy are not widely recognized, and consequently excessive examinations are frequently performed and the condition is often misdiagnosed. The clinical findings and risk factors of scurvy, including an unbalanced diet, should be better understood to ensure that treatment is initiated as soon as possible.




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