|
THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
|
Vol.117, No.8, August 2013
|
Original Article
Title
Twenty-eight Cases of Cerebrospinal Fluid Shunt Infection
Author
Chie Fukasawa and Tadashi Hoshino
Division of Infectious Diseases, Chiba Children's Hospital
Abstract
Cerebrospinal fluid (CSF) shunt infection is one of the serious complications of CSF shunting procedures. To identify the clinical features of CSF shunt infection, we retrospectively reviewed the medical records of patients who were admitted to Chiba Children's Hospital between 2005 and 2009 with a diagnosis of CSF shunt infection, which was confirmed bacteriologically.
Twenty-eight cases of CSF shunt infection in 19 patients were included in this study. Twenty-four cases of CSF shunt infection occurred within 1 year after the CSF shunting procedure, whereas some cases occurred after more than several years. Fever was noted in 21 cases, and abdominal symptoms were present in 14 cases. Although the serum C-reactive protein levels were elevated in most cases, only 13 of 27 cases in which the CSF cell count was determined developed pleocytosis in the CSF. Eight cases required more than 1 week for diagnosis, and 3 of these cases had shown abnormal findings on abdominal CT, which helped in the diagnosis. In 11 cases, the pathogen was identified only after evulsion of the shunt catheter. The most frequently isolated pathogens were Staphylococcus spp., including methicillin-resistant strains, which accounted for 71% of the cases. Removal of the colonized shunt system and shunt revision was performed in 21 cases, and 6 of these cases had a recurrence of shunt infection. On the other hand, 7 cases received only antibiotic therapy, and 4 of these cases had a relapse of shunt infection within 3 weeks. The clinical features of CSF shunt infection are different from those of the usual bacterial meningitis that occurs in healthy children, and the diagnosis of CSF shunt infection is often difficult because of non-specific symptoms and difficulty in pathogen detection. CSF shunt infection should be considered as a differential diagnosis in patients with a CSF shunt who develop fever.
|
|
Original Article
Title
The Carriage Rates and Ampicillin Resistance of Haemophilus Influenzae Type b in Infants and Young Children
Author
Akiyoshi Narai and Kentaro Iwasawa
Department of Pediatrics, Yokohama Minami Kyousai Hospital
Abstract
We analyzed the carriage rates of Haemophilus influenzae type b (Hib) in infants and young children (age, 1 month to 6 years) with lower respiratory tract infection using nasopharyngeal samples obtained annually from 2004 to 2011. The carriage rates in 2004 and 2005 were 1.2% and 1.1% (exceeding 1%), whereas those in 2006, 2007, and 2008 were 0.5%, 0.8%, and 0.2% (fluctuating at <1%), respectively. Thereafter, the carriage rate was 0.4% in 2009, when Hib vaccination was initiated, and 0.5% in 2010. However, when the free vaccination program was initiated in 2011, no Hib carriers were detected. From the results of previous publications in Japan and the present study, the Hib carriage rate in Japanese infants and young children is considered to have changed at approximately 1% for a long period. However, because of the increase in herd immunity against Hib in 2011 owing to the distribution of the Hib vaccine, a reduction in the carriage rate among infants and young children is predicted. In addition, the ampicillin (ABPC) resistance rate of 49 Hib strains detected from 2004 to 2010 was 55.1%. Of these ABPC-resistant strains, 85.2% were β-lactamase nonproducing. In the future, it is important to implement Hib vaccination to maintain herd immunity against Hib with a high ABPC resistance rate.
|
|
Original Article
Title
Clinicopathological Study of Pulmonary Arteriopathy in Patients with Trisomy 18 Complicated with Pulmonary Arterial Hypertension due to Congenital Heart Disease
Author
Masahiro Tahara1) Akane Honda1) Saiko Shimozono1) Tetsuya Nitta1) and Shigeo Yamaki2)
1)Department of Pediatrics, Tsuchiya General Hospital
2)Japanese Research Institute of Pulmonary Vasculature
Abstract
Background: Pulmonary arterial hypertension (PAH) and pulmonary vascular disease develop and progress earlier and with greater severity when complicated with congenital heart diseases (CHD) in patients with trisomy 18. The major causes of death in patients with trisomy 18 are heart failure and pulmonary hemorrhage due to CHD.
Patients and Methods: We performed open lung biopsy for PAH in 10 patients with trisomy 18 and congestive heart failure due to CHD, and analyzed the pathological findings of the small pulmonary arteries and their clinical course.
Results: The mean age at the time of the open lung biopsy was 51.4±13.0 days (range, 26-69 days). According to the Heath-Edwards classification, 1, 2, 4 and 2 patients were graded as 0, 1, 2 and 3, respectively, while 1 patient was not classifiable due to a medial defect of the small pulmonary arteries. Later pulmonary artery banding was associated with worsening obstructive pulmonary vascular disease. The values of the wall thickness (%), which is the ratio of medial thickness to the arterial external diameter, at diameters of 50 and 200 μm, were 7.8±3.2% and 4.6±1.7%, respectively. These values were lower in CHD patients with trisomy 18 than in those without chromosomal abnormalities that were reported previously (18.9±1.6% and 8.6±1.7% at diameters of 50 and 200 μm, respectively). Three patients with intimal fibrous proliferation died of heart failure and PAH, and heart-related death was preventable in 7 with intimal cellular proliferation or no intimal lesions.
Conclusions: Medial hypertrophy is mild in patients with CHD and trisomy 18, regardless of PAH severity. Earlier cardiac surgery for such patients might prevent the progression of obstructive pulmonary vascular disease and heart-related death. However, fatal vascular changes such as medial defects occur in several cases, and it remains unclear whether cardiac surgery improves the long-term prognosis of patients with trisomy 18. Therefore, physicians should discuss treatment options with the parents of patients with trisomy 18 to ensure that the selected strategy is in the best interests of the patients.
|
|
Original Article
Title
Impact of Neonatal Diseases on Childhood Mortality in Japan
Author
Satoshi Kusuda1) Masanori Fujimura2) and Hiroshi Watanabe3)
1)Maternal and Perinatal Center, Tokyo Women's Medical University
2)Osaka Medical Center and Research Institute for Maternal and Child Health
3)Teikyo University School of Medicine University Hospital, Mizonokuchi
Abstract
<Background>
Childhood deaths are occasionally caused by diseases that develop during the neonatal period. To fully characterize childhood deaths in Japan, an international comparison of childhood mortality and a survey of whether individual cases of childhood mortality are related to neonatal diseases through a review of death certificates in Japan are needed.
<Methods>
The World Health Organization Mortality Database 2010 was used for the international comparison of childhood mortality (defined as death in children aged 1-4 years) among the 19 Organization for Economic Cooperation and Development (OECD) countries. In 2005 and 2006, 1,160 and 1,085 cases of childhood mortality were registered with the National Vital Statistics in Japan, respectively. Death certificates from 1,134 and 1,054 cases, respectively, were reviewed.
<Results>
1) A linear correlation was found between neonatal and childhood mortality among the OECD countries, except for Japan. Childhood mortality in Japan was relatively higher than neonatal mortality.
2) Out of 2,118 cases of childhood mortality in Japan in 2005 and 2006, 612 cases died from diseases that had developed during the neonatal period. Among these cases, 134 cases were never discharged from the hospital before death.
3) These 134 cases could not have been saved with vigorous treatment during the neonatal period. Thus, these children might have died during the neonatal period without the level of intensive care that is performed in Japan. This level of care has come under criticism as excessive intervention. Even after re-defining these 134 deaths as being due to neonatal mortality and subtracting them from childhood mortality, neonatal mortality in Japan was still the lowest among the OECD countries, and the rank of childhood mortality in Japan improved from 14th to 13th among the 19 OECD countries.
<Conclusion>
In spite of having the lowest neonatal and infant mortality among the OECD countries, childhood mortality was relatively high in Japan. However, this difference was not attributable to the inclusion of some of the neonatal or infant deaths due to excessive treatment in neonatal intensive care units in Japan in the calculation of childhood mortality.
|
|
Original Article
Title
Impact of Establishing a Child Protection Team (CPT) in Matsudo City Hospital
Author
Kosuke Kohashi1)3) Fumitake Mizoguchi2) Tomohiko Tsuru3) Hajime Ikehara3) Tomoyasu Saito3) and Fujiko Yamada4)
1)Kamogawa City Hospital
2)Saiseikai Maebashi Hospital
3)Matsudo City Hospital Children's Medical Centre
4)Child Maltreatment Preventing Network
Abstract
We investigated the impact of a child protection team (CPT) in Matsudo City Hospital. The CPT was established in 2009. We retrospectively investigated the positive effect of establishing a CPT by comparing 2 periods: before and after CPT introduction. All data were collected using the medical charts from 1999 to 2010. We divided the cases into 2 groups: 10 years before CPT introduction and 2 years after CPT introduction. The number of cases in the former group was 10, whereas that in the latter group was 72. The 72 cases consisted of 36 cases of physical abuse, 30 cases of neglect, 2 cases of sexual abuse, and 4 cases judged as non-abusive after medical investigation. Out of 68 suspected child abuse cases, 37 cases were reported to the Child Protection Service, 14 cases were reported to the municipal office, and 17 cases were connected to the community supportive multidisciplinary network. Our investigation showed that the establishment of the CPT led to a dramatic increase in the number of suspected child abuse cases due to the increased awareness of the medical staff to child abuse through the CPT. Further development is essential, including the establishment of CPT guidelines and a performance assessment tool for the maintenance and improvement of the established CPT.
|
|
Original Article
Title
Competency Evaluation in Pediatric Ventricular Fibrillation and Shock among Doctors-in-training and Pediatricians
Author
Noriyuki Kaku Takashi Muguruma Kentaro Ide Masanori Tani and Kohei Tsukahara
Department of Acute and Critical Care, National Center for Child Health and Development
Abstract
Objective: The purpose of this study is to evaluate the competency of effectively performing pediatric resuscitation and emergency care among doctors-in-training and pediatricians, and thereby establish an optimal method for teaching pediatric resuscitation.
Methods: We evaluated 40 newly hired residents at the National Center for Child Health and Development in 2009 and 2010, 38 of whom are pediatric advanced life support (PALS) providers. The residents all participated in 2 of 7-minute simulation-based scenarios: ventricular fibrillation and shock. We measured the time to interventions and tasks required in the scenarios.
Results: In the ventricular fibrillation cases, the median time to each task and the percentage of the residents who did not perform each task were respectively as follows: ventilation (46 sec, 4%), chest compression (58 sec, 3%), defibrillation (219 sec, 15%), and epinephrine administration (353 sec, 65%). The total CPR time was 286 sec (median). In the shock cases, 55% of the residents could not complete the primary assessment. Residents tended to fail to assess the airway, neurological status, and the whole body. The percentages of the residents who could not recognize and categorize shock were 22% and 97%, respectively.
Conclusion: We found that both doctors-in-training and pediatricians were not competent in pediatric resuscitation and emergency care owing to a decline in their skills. To maintain good skills, frequent off-the-job training, such as using medical simulation, is therefore needed.
|
|
Original Article
Title
Successful Treatment of Bruck Syndrome in an Infant with Bisphosphonate
Author
Fumi Tachibana
Department of Neonatal Medicine, Ehime Prefectural Central Hospital
Abstract
Bruck syndrome is characterized by the association of bone fragility and congenital contractures resembling osteogenesis imperfecta. We report an infant with Bruck syndrome who had congenital contractures and multiple bone fractures during the neonatal period. The patient was a male infant born at term. He showed symmetrical multiple arthrogryposis at birth, but he had no other anomalies or respiratory disturbances. On day 5, his left leg became swollen, and then a left femoral fracture was discovered. He subsequently suffered repeated fractures, including a right femoral fracture, as well as ulnar and rib fractures. Low bone mineral density (BMD) in the lumbar spine and an elevated urine N-telopeptide (NTX)/creatinine (CR) ratio indicated osteopenia and bone fragility. Administration of bisphosphonate, a potent inhibitor of bone resorption, was started at the age of 1 month. During cyclic bisphosphonate therapy, his BMD increased and the NTX/CR ratio gradually decreased, and the incidence of fractures decreased. Bruck syndrome is a rare disorder and to the best of our knowledge, the present case is the first patient with Bruck syndrome in Japan who was successfully treated with bisphosphonate. Bisphosphonate administration reduces the incidence of fractures and improves joint movement.
|
|
Original Article
Title
A Case of Acute Pancreatitis in a Patient in Which Computed Tomography was Useful for Deciding Acuity
Author
Yumi Terakawa1) Tomo Sawada1) Makoto Enomoto2) Eri Hayashi2) Satoru Sakuma2) Kenji Bando2) Seiko Murakami2) and Haruo Shintaku1)
1)Department of Pediatrics, Graduate School of Medicine, Osaka City University
2)Department of Pediatrics, Izumi Municipal Hospital
Abstract
Mycoplasma pneumonia is a common complication associated with several extrapulmonary infections. Reports of pancreatitis in patients with mycoplasma pneumonia are rare. Herein, we report a case of severe acute pancreatitis in a patient with mycoplasma pneumonia. The patient was a 7-year-old girl whose condition was diagnosed as severe pancreatitis (grade 3), and who presented with vomiting, stomachache, and high blood levels of amylase during mycoplasma pneumonia treatment. Computed tomography (CT) showed inflammation extending to the inferior pole of the kidney and poor contrast enhancement in 2 pancreatic regions. Her symptoms and CT findings improved with therapy. In patients with mycoplasma infections, the possibility of concomitant complications should be considered, and these patients should be observed for changes in symptoms. CT grade is useful for deciding acuity.
|
|
Original Article
Title
A Case of Refractory Pseudomonas aeruginosa Respiratory Infection Successfully Treated with Nebulized Tobramycin
Author
Hiroshi Okada1) Naruhiko Ishiwada2) Tomohiko Tsuru1) Takashi Kigawa1) Ryugo Hiramoto1) and Isao Komori1)
1)Department of Pediatrics, Matsudo City Hospital Children's Medical Center
2)Department of Pediatrics, Graduate School of Medicine, Chiba University
Abstract
Nebulized tobramycin is considered effective for treating Pseudomonas aeruginosa infection in patients with cystic fibrosis and it has been commonly used in foreign countries. However, this therapy has rarely been practiced even for adult patients in Japan, and to the best of our knowledge, no studies have been performed on its use for pediatric patients. Therefore, we report a case wherein we used nebulized tobramycin for a neurologically impaired 5-year-old girl with refractory P. aeruginosa infection. Although the patient had tracheal separation and was undergoing long-term low-dose clarithromycin therapy, she experienced recurrent episodes of pneumonia caused by P. aeruginosa. Nebulized tobramycin therapy was initiated during the latest episode of pneumonia because it was not ameliorated by intravenous antibiotic therapy administered for more than 4 weeks.Nebulized tobramycin therapy finally ameliorated the pneumonia, and the patient was discharged. The therapy did not cause any adverse effects such as renal dysfunction and hearing disabilities, and the plasma tobramycin concentration was lower than the detectable level. Following her discharge, the patient intermittently underwent nebulized tobramycin therapy during the next 2 years. During this period, she experienced only 1 episode of pneumonia and was safely followed up as an outpatient. This experience indicates that nebulized tobramycin may be useful for treating even neurologically impaired children with refractory P. aeruginosa respiratory infection. Further studies are required to determine the clinical efficacy and long-term adverse effects of nebulized tobramycin therapy in such cases.
|
|
Original Article
Title
Erythema Nodosum Caused by Chlamydophila pneumoniae Infection without Respiratory Symptoms
Author
Atsuo Togashi1) Yuta Sasaoka1) Kenji Kondo1) Yoshiyuki Sakai1) Minami Yoda1) and Hiroyuki Tsutsumi2)
1)Department of Pediatrics, Hakodate Municipal Hospital
2)Department of Pediatrics, Sapporo Medical University
Abstract
We present the case of a 6-year-old girl suffering from erythema nodosum (EN) caused by Chlamydophila pneumoniae infection without respiratory symptoms. She was admitted to hospital with difficulty in walking owing to left pretibial exanthema with swelling and pain. Subcutaneous infection due to bacteria was suspected and antibiotics were administrated without effect. In addition, similar exanthema appeared on the right lower limb. We diagnosed her condition as EN based on appearance without performing biopsy of the lesions. There was no response to ibuprofen. Although she had had no respiratory symptoms and her chest radiograph was normal, C. pneumoniae infection was confirmed by a serologic screening test. Clarithromycin was administrated which gradually improved her condition. C. pneumoniae is a common cause of acute respiratory infection. There are some reports of EN due to C. pneumoniae, and almost all cases have respiratory symptoms, which were absent in our case. C. pneumoniae infection should be considered in patients with EN, even in the absence of respiratory symptoms.
|
|
Original Article
Title
Incontinentia Pigmenti in a Newborn with NEMO Mutation Diagnosed by Flow Cytometric Analysis of NEMO Protein
Author
Hiroko Uchio1) Takayuki Nukata1) Norihito Iba1) Daisuke Fukao1) Yukiko Hashimoto1) Yuka Tanabe1) Mihoko Inoue1) Keigo Hamahata1) Akira Yoshida1) Toru Momoi1) Tomoki Kawai2) Ryuta Nishikomori2) and Toshio Heike2)
1)Department of Pediatrics, Japanese Red Cross Wakayama Medical Center
2)Department of Pediatrics, Graduate School of Medicine, Kyoto University
Abstract
Incontinentia pigmenti is one of the neurocutaneous syndromes associated with mutations of the nuclear factor-kappaB essential modulator (NEMO) gene. The characteristic skin lesions start in four stages from or before birth, and complications in the teeth, central nervous system, and eyes are occasionally observed. An eye complication is highly critical as a cause of visual loss such that early detection and care of retinopathy should be performed. A term female infant was admitted to the NICU for erythematous eruptions on the first day of life. The characteristic skin lesions as well as histopathological examination of the skin led us to a tentative diagnosis of incontinentia pigmenti. Flow cytometric analysis of NEMO protein showed reduced NEMO protein expression in monocytes of the patient on the 14th day of life, which strengthened the diagnosis of incontinentia pigmenti caused by a mutation of the NEMO gene. Genetic analysis subsequently confirmed a mutation of the NEMO gene of the patient. Progression of retinopathy was observed and laser photocoagulation was performed on the 15th day of life. On the other hand, the number of monocytes with reduced NEMO protein expression had gradually decreased, indicating that flow cytometric analysis could be applied only in the early stage of infancy. In conclusion, flow cytometric analysis of NEMO protein can be helpful in the early diagnosis of incontinentia pigmenti, which can lead to its early detection and care for possible complications in the early stage of infancy.
|
|
Original Article
Title
Successful Treatment with Propranolol of Multiple Hepatic Hemangiomas and Associated Consumptive Hypothyroidism
Author
Hiroyuki Ikeda Ayako Sakurai Kazunori Sanayama Kazuki Furudate Hideaki Ueki Nobuyo Seimiya Yasushi Noguchi Shunji Igarashi and Shosuke Sunami
Department of Pediatrics, Narita Red Cross Hospital
Abstract
We report the case of a 2-month-old girl with multiple hepatic hemangiomas and associated consumptive hypothyroidism. She presented with heart murmur, jaundice, abdominal distention, and multiple cutaneous hemangiomas. Blood examination revealed hypothyroidism, and ultrasonography showed multiple hepatic hemangiomas. Propranolol administered orally at 2 to 3 mg/kg per day induced regression in 6 weeks. High-dose L-thyroxine replacement therapy was initially needed, but the dose could be reduced as the hemangiomas regressed. This case highlights the importance of identifying hypothyroidism in patients with multiple hemangiomas and shows that multiple hepatic hemangiomas can be successfully treated with propranolol.
|
|
Original Article
Title
A Case of Eosinophilic Esophagitis
Author
Kyoko Takayanagi1) Yoshiko Nakayama1)2) Yuri Etani1) Kie Nakao1) Koji Kawamoto1) and Shinobu Ida1)
1)Department of Pediatric Gastroenterology and Nutrition, Osaka Medical Center and Research Institute for Maternal and Child Health
2)Department of Pediatrics, Shinshu University School of Medicine
Abstract
During the last decade, clinical practice has seen a rapid increase in the number of patients with esophageal eosinophilia who were thought to have gastroesophageal reflux disease (GERD) but who failed to respond to medical or surgical GERD management. Subsequent studies demonstrated that these patients had a "new" disease termed eosinophilic esophagitis (EoE). We report a typical EoE case involving a 13-year-old boy who presented with typical upper gastrointestinal endoscopic/histologic findings and who was successfully treated with oral corticosteroids. He was referred to our department for detailed study and treatment of upper abdominal pain possibly due to allergic disorder. He showed other symptoms such as upper abdominal pain, heartburn, and food impaction. Blood examination showed high levels of peripheral blood eosinophils and total IgE. Upper gastrointestinal endoscopic examination was performed with a suspected diagnosis of eosinophilic gastroenteritis, revealing linear fissures, circular rings, whitish rough mucosa, and loss of esophageal vascularity, sparing the stomach and duodenum. Histologic examination of the esophageal biopsy specimens revealed marked mucosal eosinophilic infiltration (more than 20 eosinophils per high power field, 400×). The patient's condition was diagnosed as EoE on the basis of the marked esophageal eosinophilia observed in the biopsy specimens. Although an oral proton pump inhibitor (PPI) had been prescribed, his symptoms did not improve. However, oral prednisolone improved both his symptoms and endoscopic findings. EoE in children is very rare and few cases have been reported in Japan. As there is an increasing number of children presenting with allergic diseases, we highly recommend active endoscopic examination to differentiate EoE in patients having GERD-like symptoms that are unresponsive to PPI therapy.
|
|
Title
Thigh Injections for Simultaneous Vaccination
Author
Masahiko Kimura
Kimura Children and Family Clinic
Abstract
We introduced the option of subcutaneous injection into the anterolateral aspect of the thigh for simultaneous vaccination. Of 75 caregivers (90%) in whom we provided an explanation using a prepared illustration, 67 accepted the thigh injections for their children. Out of 125 children, we performed the subcutaneous injection in 69 children aged from 2 months to 22 months between November 2011 and July 2012. After the vaccination, we asked the caregivers for their feedback regarding the thigh injections. Of 65 caregivers, 7 (11%) indicated an unusual feeling because it was their first time to have thigh injections for their children. However, 63 of the 65 caregivers (95%) answered that they would allow thigh vaccination again. In 2 children, swelling of the entire lower extremities on the injected side developed, but it subsided the following day. Our experience shows that thigh injections can be smoothly introduced for simultaneous vaccination.
|
|
|
Back number
|
|
