gakkaizashi


THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.117, No.7, July 2013


1. Characteristics of Childhood Urinary Tract Infection in Saitama City Hospital from 2001 to 2010
2. Glomerular Hypertrophy and Childhood Proteinuria in Patients Born with Extremely Low Birth Weight
3. A Nationwide Survey of Infants Continuously in the Neonatal Intensive Care Unit for More than One Year
4. Analysis of Cases Admitted to the Pediatric Intensive Care Unit Following Laryngomicrosurgery for Laryngomalacia or Laryngotracheoesophageal Cleft
5. Clinical Evaluation of 50 Hospitalized Infants and Young Children with Mild Traumatic Head Injury
6. Clinical and Therapeutic Problems Regarding Adrenoleukodystrophy in Japan
7. Hopkins Syndrome Associated with Enterovirus 71 Infection
8. A Case of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Found upon Performing Further Examination of Hematuria
9. Food Protein-induced Enterocolitis Syndrome: Successful Diagnosis by Abdominal Ultrasonography in three Infants
10. Hemothorax and Pericardial Defect Caused by an Isolated Costal Exostosis
11. A Case of Intramural Duodenal Hematoma Following Upper Gastrointestinal Endoscopy in a Child


Original Article
Title
Characteristics of Childhood Urinary Tract Infection in Saitama City Hospital from 2001 to 2010
Author
Munehiro Furuichi Akari Arakawa Yuko Hamahata Machiko Usui Motoko Shimoyamada Megumi Yamada Masayuki Akashi Kyoko Kudo and Seiji Sato
Department of Pediatrics, Saitama City Hospital
Abstract
From January 2001 through December 2010, 166 children (120 boys) were diagnosed as having urinary tract infection (UTI) and were admitted to our hospital. Of these 166 children, 48 children (29%) were younger than 3 months, and 135 children (81%) were younger than 1 year. The main causative agents among 158 children with microbiologically proven UTI were Escherichia coli (125/158), Enterococcus faecalis (14/158) and Klebsiella pneumonia (5/158). An imaging study for the assessment of vesico-ureteral reflux (VUR) was performed in 157 cases which revealed unilateral VUR in 36/157 (23.0%) cases and bilateral VUR in 22/157 (14.0%) cases. Higher grades of VUR were associated with lower rates of spontaneous resolution of VUR.




Original Article
Title
Glomerular Hypertrophy and Childhood Proteinuria in Patients Born with Extremely Low Birth Weight
Author
Asako Hayashi Yoko Santo and Kenichi Satomura
Department of Pediatric Nephrology and Metabolism, Osaka Medical Center and Research Institute for Maternal and Child Health
Abstract
Of late, the risk of hypertension and micro-albuminuria caused by a decreased number of glomeruli is increasing in adult patients with low birth weight (LBW). We report 5 cases of extremely low birth weight (ELBW) children who developed proteinuria at a school-going age. These children were born at 23-25 weeks of gestation, and their birth weight was in the range of 532-732 g. Proteinuria was detected when they were 6-15 years of age in a routine urinalysis performed as part of a routine medical check-up at the children's schools. Renal biopsy revealed a diffuse increase in glomerular size, without mesangial proliferation or glomerular sclerosis. We speculate that children with ELBW develop proteinuria in early childhood because they have less number of glomeruli than those with LBW. Furthermore, the increase in the number of patients with childhood proteinuria could be due to the increase in the number of ELBW survivors as a result of medical advances.




Original Article
Title
A Nationwide Survey of Infants Continuously in the Neonatal Intensive Care Unit for More than One Year
Author
Satoshi Kusuda1) Fumika Yamaguchi1) and Masanori Tamura2)
1)Tokyo Women's Medical Center, Maternal and Perinatal Center
2)Saitama Medical Center
Abstract
Background
As neonatal intensive care progresses, more severely debilitated and premature infants are surviving than ever before in Japan. On the other hand, the survival of these critically ill infants is causing a shortage of neonatal intensive care unit (NICU) beds, as they require prolonged stays for treatment. To determine the appropriate measures that these infants require, a nationwide survey that investigates not only the incidence of infants who require a prolonged stay in the NICU but also their final outcomes is an essential first step.
Methods
From 2008 through 2010, a questionnaire on infants who required a prolonged stay in the NICU was sent to 206 hospitals who are members of the Japanese Neonatologist Association. A "prolonged stay" was defined as treatment in the NICU including its step down units for more than 1 year. The number of infants with a prolonged stay, the occurrence of infants with a prolonged stay per year, and the outcomes of these infants, along with the number of NICU beds and the total number of infants admitted were collected and analyzed.
Results
Of the 206 hospitals surveyed, 151 responded to the questionnaire. According to the responses, 744 infants who were considered prolonged stay patients were registered in these hospitals. Each year, it is estimated that about 200 infants become prolonged stay patients in Japan. The number of prolonged stay NICU patients peaked in 2006 and has gradually decreased since then. Of the infants whose prolonged stays could be attributed to a physical condition or disease, 226 were diagnosed as having congenital anomalies, 191 had very low birth weights, 142 had experienced severe birth asphyxia, 101 were diagnosed as having chromosomal anomalies, 40 had neuromuscular disorders, 15 had congenital heart disease, 6 had infections, and 23 had other disorders. The final outcomes of 652 infants were recorded. At 2 years of age, 30% had been discharged from the NICU, 20% transferred to another ward or facility, 20% had died, and the remaining 30% remained in the NICU. At 3 years of age, 15% of the infants were still in the NICU and at 4 years of age, 10% remained. The proportion of infants with congenital anomalies or low birth weights gradually decreased as the infants' age increased, whereas the proportion of infants with birth asphyxia increased.
Conclusion
Even though the number of infants who require a prolonged stay in the NICU has decreased gradually over the past several years, almost 200 infants in Japan require prolonged treatment in these specialized neonatal units each year. Thus, the local system to support these infants discharged from the NICU must increase their capacity to accommodate about 60 infants each year to meet the current demand.




Original Article
Title
Analysis of Cases Admitted to the Pediatric Intensive Care Unit Following Laryngomicrosurgery for Laryngomalacia or Laryngotracheoesophageal Cleft
Author
Yo Okizuka Tatsuya Kawasaki Taku Koizumi Yuichiro Muto Hiroshi Kurosawa Ryosuke Fukushima Takuma Kishimoto Takuya Miya Ko Matsui Tokio Wakabayashi Yusuke Ito Hatsuka Minamino Takamori Kanazawa and Ikuya Ueta
Department of Pediatric Critical Care, Shizuoka Children's Hospital
Abstract
Children undergoing laryngomicrosurgery for laryngomalacia and laryngotracheoesophageal cleft should remain electively intubated in the pediatric intensive care unit (PICU) for several days following surgery to facilitate wound healing and prevent upper airway obstruction. Moreover, these patients require postoperative sedation and analgesia with or without neuromuscular blockade in the PICU. We performed 11 surgeries (8 patients; age, 1-36 months; median age, 7 months) during the period between June 2007 and May 2011. Of the 8 patients, 5 (8 surgeries) had cardiac or central nervous system disorders. A combination of midazolam and opioid analgesics (e.g., fentanyl and morphine) was administered to all patients, and 10 cases were also administered additional sedatives. Neuromuscular blockade was induced in only 1 patient. The intubation duration was 52-166 hours (median duration, 95 hours). During this period, 4 patients suffered from mild atelectasis and 1 patient experienced mild withdrawal syndrome. Successful extubation was achieved at the first trial in 9 cases, whereas the others had successful extubation after further surgery. All patients achieved successful extubation without severe complications, and good postoperative progress in the PICU was shown by all 8 patients.




Original Article
Title
Clinical Evaluation of 50 Hospitalized Infants and Young Children with Mild Traumatic Head Injury
Author
Nao Aoki Yi Zhu and Yuji Koike
Department of Pediatrics, Disaster Medical Center
Abstract
Mild traumatic head injury (mTHI) is one of the most common injuries among infants and young children. However, the clinical features of this injury in Japanese children have not yet been fully elucidated. We performed a retrospective medical record review of 50 children with mTHI who were less than 4 years of age (30 boys and 20 girls) and were admitted to our hospital between January 2005 and September 2010. Their mean age was 15.5 months, and the most common cause of mTHI was a fall (33/50, 66.0%). In most cases, mTHI occurred at home because of the carelessness of caretakers. Among 47 patients who had undergone brain computed tomography (CT), 21 (44.7%) had skull fractures (SF) and 16 (34.0%) had intracranial hemorrhage (IH); however, no neurosurgical interventions were needed. All patients were discharged from the hospital without any sequelae.
Caretakers should be aware of the possibility of mTHI occurring at home, and this awareness may in turn reduce the incidence of mTHI. Furthermore, clinicians should take note of clinically important conditions, such as SF and IH, in patients with mTHI. A guideline on performing brain CT for mTHI among infants and young children is needed in Japan.




Original Article
Title
Clinical and Therapeutic Problems Regarding Adrenoleukodystrophy in Japan
Author
Syun Ichikawa1) Masayuki Shimono1) Masahiro Ishii1) Ayako Senju1) Kaoru Sato1) Yuko Honda1) Ryosuke Miyaji1) Reiko Saito1) Yukiyo Yamamoto1) Nobuyuki Shimozawa2) and Koichi Kusuhara1)
1)Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health
2)Life Science Research Center, University of Gifu, School of Medicine
Abstract
Adrenoleukodystrophy (ALD) is an X-linked inherited peroxisomal enzyme disorder. The disease is believed to be clearly related to a problem in the metabolism of saturated very long chain fatty acids (VLCFA). A boy, whose condition was diagnosed as ACTH unresponsiveness since he was 8, was brought to our hospital at the age of 11 years. By the age of 12, he began to occasionally show communication problems, and a worsening paralysis of the right hand. The MRI scan indicated a type of leukodystrophy, and his serum VLCFA levels were 10 times higher than normal. These results clearly indicated ALD. We attempted treatment with unrelated cord blood stem cell transplantation, but the results were unsuccessful. Diagnosing ALD patients during the neurologically asymptomatic stages is very important in order to procide timely and effective stem cell therapy. Thus, we believe that it is essential to test serum VLCFA for all boys who show insufficient adrenal gland functions, and for any male siblings of ALD patients to receive the same serum test, or at least the families should consult with their counselor on this specific issue, even if the boys in question are asymptomatic. To this end, we recommend the establishment of a national counseling network for information regarding ALD.




Original Article
Title
Hopkins Syndrome Associated with Enterovirus 71 Infection
Author
Kotoko Sugaya1) Shin-ichiro Hamano1) Ryuki Matsuura1) Kenjirou Kikuchi1) Manabu Tanaka1) and Motoyuki Minamitani2)
1)Division of Neurology, Saitama Children's Medical Center
2)Department of Child Health and Human Development, Saitama Children's Medical Center
Abstract
We describe the case of a 2-year-old boy who developed flaccid paralysis of the left upper limb 1 week after an asthmatic attack. His condition was diagnosed as subluxation of the radial head at an orthopedic clinic. As his condition persisted, the patient visited another hospital where several examinations including brain magnetic resonance imaging (MRI) scan were performed; however, a confirmatory diagnosis was not achieved. Three months later, the patient was admitted to our hospital, with a history of paralysis. The patient underwent T2-weighted MRI of the spinal cord which revealed a focal high-intensity area in the left anterior horn at the C3 to C6 levels. Moreover, Enterovirus was identified in the patient's stool specimens, and the serum titer for Enterovirus 71 was significantly increased. On the basis of our findings, a diagnosis of Hopkins syndrome associated with Enterovirus 71 infection was made. In conclusion, clinicians who treat pediatric asthmatic bronchitis or pediatric orthopedic conditions should strongly consider Hopkins syndrome when flaccid paralysis is evident, with an abrupt onset after an attack of asthmatic bronchitis.




Original Article
Title
A Case of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Found upon Performing Further Examination of Hematuria
Author
Hiroaki Kanai Hiroki Sato and Yoshichika Takei
Department of Pediatrics, Suwa Central Hospital
Abstract
A 14-year-old girl who had no history of dysmenorrhea after reaching menarche (age, 12 years) was referred to our hospital for further examination after being detected positive for urinary occult blood during urinalysis. Early abdominal ultrasonography revealed right renal agenesis and the presence of 2 uterine fundi; abdominal and pelvic MRI showed uterus didelphys, atresia of the right hemivagina, hematocolpos, and right renal agenesis, leading to a diagnosis of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). To preserve the patient's fertility and prevent infection, minimally invasive fenestration was performed by the transvaginal approach. The postoperative course was good, and the urine occult blood disappeared. Although the patient had only 1 kidney, her renal function remained normal. The OHVIRA syndrome is a rare congenital uterine malformation and is often discovered in the case of dysmenorrhea after menarche, persistent lower abdominal pain, or a lower abdominal mass. However, the disorder has broad-spectrum clinical presentations and symptoms may be completely absent, as in this patient. Unless such possibilities are considered beforehand, the OHVIRA syndrome may not always be easy to diagnose. Furthermore, if it is not accurately diagnosed and the chronic uterine inflammation is left uncontrolled, it may result in the deterioration of fertility, leaving no alternative but to perform an invasive surgical procedure. Thus, early diagnosis and treatment, and regular postoperative follow-up must be conducted. Additionally, because this condition involves unilateral renal agenesis, it is important that the renal function be evaluated on a regular basis. In cases where congenital uterine malformation is suspected, the patients must be examined for urinary system abnormalities.




Original Article
Title
Food Protein-induced Enterocolitis Syndrome: Successful Diagnosis by Abdominal Ultrasonography in three Infants
Author
Keisuke Jimbo1) Kenji Hosoi1) You Aoyagi1) Tohru Fujii1) Takahiro Kudo1) Yoshikazu Ohtsuka1) Toshiaki Shimizu1) Nobuyoshi Asai2) and Tatsuo Kohno3)
1)Department of Pediatrics and Adolescent Medicine, Juntendo University Graduate School of Medicine
2)Depertment of Ultrasonoraphy and Diagnosis, Ibaraki Children's Hospital
3)Depertment of Radiology, Tokyo Metropolitan Children's Medical Center
Abstract
The prevalence of food protein-induced enterocolitis syndrome (FPIES) has been increasing in recent years. In the neonatal or infantile period, patients with FPIES may present with gastrointestinal disorders after breast milk or formula milk ingestion. However, because of the lack of conclusive laboratory tests and pathognomonic histologic changes, it is considered difficult to diagnose FPIES. We evaluated the clinical findings and the gray scale and color Doppler sonograms of 3 infants diagnosed as having FPIES who presented the classical gastrointestinal symptoms and the clinical course of FPIES. We found thickening and swelling of the intestinal wall, poor peristalsis, and thickening and an increase in the blood flow in the mesentery of the small intestine in both the acute phase and the post challenge test, which disappeared with the avoidance of formula milk. Furthermore, vessel density, measured to quantify the mesenteric blood flow in the acute phase, showed a higher value than the cutoff points. We therefore suggest that ultrasonography can be a useful tool for the diagnosis of FPIES.




Original Article
Title
Hemothorax and Pericardial Defect Caused by an Isolated Costal Exostosis
Author
Taro Watanabe1) Nobuo Ooyama1) Kenichiro Takahashi1) Takayoshi Kyoda1) Yoshito Kamio2) Takashi Soga1) Shigeru Uemura3) and Yoh Umeda1)
1)Showa University Northern Yokohama Hospital Children's Medical Center
2)Showa University Northern Yokohama Hospital Respiratory Center
3)Showa University Northern Yokohama Hospital Cardiovascular Center
Abstract
Introduction
Exostosis is a common benign bone tumor in children. If it occurs in the ribs, it may cause chest pain but may rarely result in hemothorax. We report a case of hemothorax caused by an isolated costal exostosis.
Case report
A 14-year-old boy was admitted to our hospital with a history of sudden left-sided chest pain and mild dyspnea. Computed tomography (CT) revealed left hydrothorax. Three-dimensional (3D) image reconstruction showed a short bony spicule arising from a left third rib projecting inward toward the lung. Thoracoscopy confirmed left costal exostosis along with a pericardial defect at the site of contact with the exostosis. There was no hemorrhage from the pleura, diaphragm or around the heart. Thoracoscopic resection of the exostosis was performed and the patient's condition resolved without any complication. No other exostosis was found elsewhere in the body.
Conclusion
Costal exostosis is a rare cause of hemothorax in children and should be considered among the possible etiologies during work up. Although conventional CT may be helpful for diagnosis, 3D image reconstruction is necessary for definitive diagnosis.




Original Article
Title
A Case of Intramural Duodenal Hematoma Following Upper Gastrointestinal Endoscopy in a Child
Author
Kazumasa Ogura Taisuke Ohto Kasumi Okushima Yukiko Mori Yasuhiro Watanabe and Yoshihiro Taniguchi
Department of Pediatrics, Fukui Red Cross Hospital
Abstract
We report a case of intramural duodenal hematoma following upper gastrointestinal endoscopy that caused duodenal obstruction and was resolved with conservative management. A 9-year-old boy underwent upper gastrointestinal endoscopy under general anesthesia for the evaluation of a stomachache, loss of appetite, and weight loss. After 1.5 hours, the patient presented with epigastric pain and frequent bilious vomiting. Duodenal stenosis due to the presence of an intramural hematoma of the horizontal portion of the duodenum was confirmed using abdominal imaging techniques. Although we monitored the patient's condition carefully, this accidental complication occurred. The hematoma gradually regressed by conservative treatment. The patient completely resumed oral intake within several weeks and recovered uneventfully. The development of intramural duodenal hematomas in children without any underlying diseases (e.g., bleeding disorders) is mostly observed following blunt abdominal trauma. In this case, periduodenal adipose tissue reduction due to sudden weight loss and the performance of endoscopic maneuvers under general anesthesia were regarded to be risk factors for hematoma onset.




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