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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.119, No.8, August 2015
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Original Article
Title
Epidemiological and Clinical Characteristics of the 2013 Hand, Foot and Mouth Disease Outbreak
Author
Takafumi Matsuoka1) Akiko Matsuoka1) Itsuo Matsuoka1) and Yuji Inaba2)
1)Matsuoka Pediatric Office
2)Department of Pediatrics, Shinshu University School of Medicine
Abstract
There was an outbreak of hand, foot and mouth disease (HFMD) during the summer of 2013, after a gap of two years, around Matsumoto, Nagano Prefecture. The epidemic started in the 25th week and reached a peak in the 28th week at our clinic. There were 273 children affected by the 52nd week. The epidemiological and clinical symptoms of this HFMD outbreak changed after the middle of the season. A search for the causative virus eventually revealed that before the 28th week, there was a single epidemic of Enterovirus 71 (EV71) which eventually peaked and evolved to a mixed epidemic since Coxsackievirus A6 (CA6) was simultaneously detected after the 29th week along with EV71. In addition, comparison of the clinical characteristics of the HFMD outbreak due to the two viruses showed that the CA6 infection was contracted by a younger age group and was accompanied by a high fever compared with EV71. In the CA6 infection, the rashes were accompanied by large blisters and were observed over an extensive region, including the face and body, apart from the extremities, and changes in the nails included nail detachment.
The onset of this new HFMD outbreak presented an important opportunity to understand the diversity of Enterovirus and suggested the need for consideration of different infection control measures in medical and educational institutes.
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Original Article
Title
Need for a Pediatric Emergency System for Status Epilepticus in Saitama Prefecture
Author
Kenjiro Kikuchi1)2) Shin-ichiro Hamano1) Erica Hiwatari1) Yuko Hirata1)2) Atsuko Oba1) Yuji Kumagai1) Reiko Koichihara1) Manabu Tanaka3) Motoyuki Minamitani1) and Hiroyuki Ida2)
1)Division of Neurology, Saitama Children's Medical Center
2)Department of Pediatrics, Jikei University School of Medicine
3)Department for Child Health and Human Development, Saitama Children's Medical Center
Abstract
Objective: This study sought to ascertain the present need for a pediatric emergency system for childhood status epilepticus (SE) in Saitama prefecture.
Methods: We retrospectively reviewed the medical records of children with SE registered at our institution. SE was defined as any seizure lasting more than 30 min or any intermittent seizure lasting more than 30 min from which the child did not regain consciousness. We investigated age at onset of SE, etiology, duration of SE, and the time required for transportation of SE patients by ambulance.
Results: A total of 156 SE episodes in 122 children (61 boys, 61 girls; median age, 2.9 years) were reviewed. The etiologies of SE were epilepsy (48.7%), febrile seizure (34.6%), acute encephalopathy/encephalitis (11.5%), and other (5.1%). The median duration of SE was 60 min (range, 30-5,760 min), with SE lasting more than 60 min in 56 episodes (35.9%). Generalized seizure occurred in 83 episodes (53.2%) and partial seizure in 73 episodes (46.8%). Episodes of SE lasting more than 60 min were more frequent for partial seizure than for generalized seizure (36 episodes vs 20 episodes, p=0.001). The mean (± standard deviation) time from emergency call to transport to our institution by ambulance was 41.1± 14.9 min, which is similar to the national average of 38.7 min.
Conclusions: The data indicate that it may take time to decide the receiving hospital added to the time for actual transport. There is an urgent need to establish a pediatric emergency system, which should include pre-hospital treatment, for pediatric SE to reduce the time taken for transportation by ambulance.
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Case Report
Title
Successful Control of Idiopathic Dilated Cardiomyopathy in an Infant after the Reintroduction of a Low-dose Carvedilol Therapy with an Inotropic Agent
Author
Yasuo Suzuki1) Seigo Okada1) Tsuyoshi Takegawa1) Shunji Hasegawa1) Shouichi Ohga1) and Etsuko Tsuda2)
1)Department of Pediatrics, Yamaguchi University Graduate School of Medicine
2)Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center
Abstract
Dilated cardiomyopathy (DCM) often causes fatal heart failure in children. Recently, the clinical outcome of pediatric DCM has improved by treatment with angiotensin-converting enzyme inhibitors (ACEI) and beta-blockers. However, administration of beta-blockers is challenging in infants with heart failure. There is little information about the optimal management of DCM using beta-blockers.
We report a case of an infant with idiopathic infantile DCM who was successfully treated with a phosphodiesterase type 3 inhibitor, digoxin and very low-dose carvedilol.
The patient was hospitalized at age 6 months because of failure to thrive and respiratory distress. Initially, administration of diuretics and ACEI appreciably controlled the progressive heart failure. We commenced carvedilol with 0.04 mg·kg-1·day-1 on day 83 after admission. During the up-titration of carvedilol, heart failure worsened on day 230. We stopped carvedilol, and began intravenous administration of milrinone and diuretics. Oral digoxin and pimobendan were added. The brain natriuretic peptide (BNP) levels decreased to 200 pg/mL, We reintroduced carvedilol at 0.002 mg·kg-1·day-1 on day 365. The dose was increased every 2 weeks by 0.001 mg·kg-1 while monitoring the plasma BNP level. After heart failure was controlled, on day 850, she was discharged from the hospital. The left ventricular ejection fraction on two-dimensional echocardiogram increased to 60%, and the New York Heart Association classification improved to class 1 when she was 4 years old.
Step-by-step dose escalation of carvedilol with an inotropic agent could be useful for prolonged control of DCM in infancy.
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Case Report
Title
Usefulness of the Muscle Repetitive Stimulation Test for the Diagnosis of Infantile Botulism
Author
Sosuke Yoshikawa1) Hiroshi Miyazaki1) Naohisa Kawamura1) and Hiroshi Tamai2)
1)Department of Pediatrics, Osaka Rosai Hospital
2)Department of Pediatrics, Osaka Medical College
Abstract
This report describes the case of a 6-month-old boy with infantile botulism, the 31st reported case of infantile botulism in Japan. He was admitted to our hospital with a 1-week history of constipation, poor sucking, and flaccid paralysis. On examination, he showed severe generalized muscle weakness, ptosis, and absent deep tendon reflexes of the limbs. Blood tests, cerebrospinal fluid examination, brain imaging, and electroencephalography showed no abnormalities. On the 5th hospital day, neuromuscular junction disease was suspected, and the 3 Hz/min muscle repetitive stimulation test was performed. The test confirmed the waning phenomenon, and steroid pulse therapy was started based on an initial diagnosis of the generalized type of myasthenia gravis. However, his symptoms failed to improve, and respiratory tract obstruction caused by sputum led to respiratory arrest. The patient required intubation and mechanical ventilation for resuscitation. Infantile botulism was suspected because the second course of steroid pulse therapy was ineffective. Stool examination was positive for botulinum toxin on the 21st hospital day. The patient was maintained on supportive therapy, and he gradually recovered from infantile botulism. He was extubated on the 32nd and discharged from hospital on the 75th hospital day. Although his mother had been eating honey every day starting 1 week before the onset of infantile botulism, no botulinum spores were detected in the honey; therefore, no identifiable source of infection was found. We suggest that the muscle repetitive stimulation test is useful for the diagnosis of infantile botulism.
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Case Report
Title
Successful Rescue of Graft Failure by Second Allogeneic Cord Blood Transplantation with Reduced Intensity Conditioning in a Patient with CD40 Ligand Deficiency
Author
Atsumi Hanawa Yoji Sasahara Tojyo Nakayama Kunihiko Moriya Yuko Watanabe Yuka Nanjyo Masahiro Irie Hidetaka Niizuma Takesi Rikiishi and Shigeo Kure
Department of Pediatrics, Tohoku University Graduate School of Medicine
Abstract
We report a patient who suffered from oral ulcer and recurrent infections at the age of 4 months in whom CD40 ligand deficiency (hyper-IgM syndrome type I) was diagnosed. Although he had been in good clinical condition by immunoglobulin replacement therapy and prophylactic antibiotics, he developed acomplicated autoimmune disorder with fever and refractory stomatitis at the age of 9 years. Steroid therapy was used to control the autoimmune disorder. To minimize long-term side effects, we treated him with reduced intensity conditioning (RIC) consisted of fludarabine, melphalan, low-dose total body irradiation (TBI) and subsequent allogeneic cord blood stem cell transplantation (CBSCT) as curative therapy, because he had no suitable HLA-matched bone marrow donor. The first CBSCT resulted in primary graft failure on day 32. After spontaneous recovery of recipient hematopoiesis, we performed RIC consisting of fludarabine, cyclophosphamide, low-dose TBI and a second allogeneic CBSCT. We achieved an engraftment on day 19 and complete chimera of donor-type without any serious regimen related toxicity (RRT). Although he developed chronic graft-versus-host disease, he has been doing well without immunoglobulin replacement for 22 months since the second transplantation. We speculated that the intensity of our first RIC regimen might not be enough to achieve engraftment in unrelated HLA-mismatched CBSCT. A second RIC and CBSCT resulted in successful engraftment with reduction in rejection capacity, possibly due to genomic instability of recipient T cells after first conditioning, as demonstrated by the absence of anti-HLA antibody or autoimmune disorder. More patients are needed for further analysis to conclude optimal conditioning regimen to achieve stable engraftment, complete chimera and less RRT in patients with CD40 ligand deficiency.
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Case Report
Title
Two Patients with Childhood-onset Pseudohypoaldosteronism Type II with Gene Mutation Identified
Author
Keiko Nagahara1) Yoshifusa Abe1) Akira Hojo1) Kazushige Dobashi1) Yoshie Nakamura2) Masahiro Goto2) Motoko Moriyama3) Kiyoshi Isobe3) Shinichi Uchida3) Yukihiro Hasegawa2) and Kazuo Itabashi1)
1)Department of Pediatrics, Showa University School of Medicine
2)Endocrinology and Metabolism Division, Tokyo Metropolitan Children's Medical Center
3)Department of Nephrology, Tokyo Medical and Dental University
Abstract
Pseudohypoaldosteronism type II (PHAII) is characterized by hyperkalemia, hyperchloremia, and metabolic acidosis owing to hyperactivity of the Na-Cl cotransporter (NCC) in the distal tubules. The involvement of four genes has been reported: With-no lysine [K] (WNK1, WNK4), Kelch-like 3 (KLHL3), and Cullin3 (CUL3). Only two child-onset PHAII patients, in whom gene mutations were identified, have been reported thus far. Here, we report two additional cases. Case 1 was a 6-year-old boy. He had short stature from infancy. At school starting age, it was revealed that he had hyperkalemia, hyperchloremia, metabolic acidosis, short stature, hypertension, and decayed teeth. Familial genetic analysis revealed that he had de novo mutation in CUL3 Intron 9. Case 2 was a 21-year-old woman. At the age of three years, her father was given a diagnosis of PHAII, and she had hyperkalemia, hyperchloremia, and metabolic acidosis. She exhibited no physical abnormalities. Genetic analysis revealed that she and her father carried a KLHL3 mutation (S433N). In both patients, levels of blood renin and aldosterone were normal and the results of the tolerance tests were consistent with PHAII. Their subsequent courses were controlled by salt restriction and oral thiazide treatment. In conclusion, PHAII should be considered in the differential diagnosis, when the patient presents hyperkalemia, hyperchloremia, and metabolic acidosis. To prevent complications caused by metabolic acidosis, gene analysis is not only useful for definitive diagnosis but also important in the early treatment of PHAII.
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Case Report
Title
Acute Neonatal Appendicitis Diagnosed by Enhanced Computed Tomography Prior to Surgery
Author
Hirotaka Miyazawa1) Kazuko Sugai1)2) Yumiko Miyaji1) Yoshinori Kobayasi1) Ayako Fukuyama1) Youichi Kaburaki1) Takeki Shiina3) and Masato Shinkai4)
1)Department of Pediatrics, National Hospital Organization Yokohama Medical Center
2)Department of Pediatrics, National Hospital Organization Fukuyama Medical Center
3)Department of Radiology, National Hospital Organization Yokohama Medical Center
4)Department of Surgery, Kanagawa Children's Medical Center
Abstract
Acute appendicitis in neonates is very rare with only 41 cases recorded in Japan over the past 87 years. None were diagnosed before surgery. We report a case of acute neonatal appendicitis diagnosed by contrast enhanced computed tomography imaging before emergency surgery.
An 18-day-old boy presented with fever and moaning in the ER and was hospitalized. A bacterial infection was suspected because of a high CRP level and procalcitonin level. Initially he was diagnosed with severe sepsis because his mother was positive for Group B Streptococcus (GBS) infection at pregnancy, and was treated with antibiotics. A few hours after hospitalization, however, he developed gastromegaly and vomiting. We therefore concluded that the focus of inflammation was in the abdomen and performed contrast enhanced computed tomography (CT). The CT image showed appendicitis. An operation was performed the same day and revealed a perforated appendix and diffuse peritonitis. His postoperative course was uneventful.
We suggest, therefore, that a diagnosis of appendicitis should be considered when babies present with apparent bacterial infection, even during the neonatal period and enhanced computed tomography is useful for acute neonatal appendicitis.
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