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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.121, No.12, December 2017
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Original Article
Title
The Importance of Multidisciplinary Support for Pediatric ICD Patients
Author
Yuki Shiomi Masaru Yamakawa Chie Aota Chisato Miyakoshi and Satoru Tsuruta
Department of Pediatrics, Kobe City Medical Center General Hospital
Abstract
Implantation of an implantable cardioverter defibrillator (ICD) in pediatric patients who have the risk of ventricular arrhythmia is much less than in adults. In our hospital, ICD implantation was performed in five patients at the mean age of 15.4 (13-21) years old and all are surviving without neurological deficit. Three of them developed psychological problems such as anxiety, depression, suicidal ideation, conflict with exercise restriction, trouble sleeping, and self-denial with or without device problems, however they all have returned to school or work life through medical and mental support by our multidisciplinary team. ICD implantation in pediatric patients will increase because of its efficacy for ventricular arrhythmia, whereas there are many problems, such as complexity of device settings in growing patients, deep fears of ICD shock, and exercise restriction in their otherwise physically active school life. Not only medical but psychological and educational support for pediatric ICD patients forging self-identity are essential, but still insufficient. We should keep multidisciplinary support for pediatric ICD patients in mind.
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Original Article
Title
Antibiotic Coverage against Pneumonia with Pseudomonas Aeruginosa Recovered from Tracheal Aspirates in Children
Author
Eiki Ogawa1) Kensuke Shoji1) Koichi Mizuguchi2) Mitsuru Kubota2) and Isao Miyairi1)
1)Division of Infectious Diseases, National Center for Child Health and Development
2)Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development
Abstract
Pediatric patients with tracheostomy often require hospitalization for pneumonia. However, microbiological diagnosis is often difficult. In such cases Pseudomonas aeruginosa is often recovered from tracheal aspirates, which often leads to use of prolonged broad spectrum antibiotics. We sought to investigate the use and the necessity of antipseudomonal antibiotics in our hospital. This retrospective study included patients aged 0-18 years old with tracheostomy, who required hospitalization for pneumonia from April 2012 to August 2015. Forty-nine cases were diagnosed as pneumonia during the period, and the median age was 6 years old (male 41%). P. aeruginosa was detected in 38 cases (78%) from tracheal aspirate cultures. Thirty-three cases received empiric antibiotics without antipseudomonal activity of which, 29 (88%) completed therapy with the same antibiotics. The four cases who required change to antipseudomonal antibiotics recovered and none were fatal. P. aeruginosa detected from tracheal aspirates may not be a true pathogen of pneumonia in children with tracheostomy.
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Original Article
Title
Carnitine Deficiency Risk in Patients Receiving Tubal Ingestion: Comparison between Intragastric and Intrajejunal L-carnitine Administration Routes
Author
Chika Ueno1) Miyoko Imayoshi1) Goro Yokota1) Shinichiro Oki1) Shuichi Aramaki1) Kyouko Tashiro2) Takahiro Inokuchi2) and Shuichi Yamamoto1)
1)Department of Pediatrics, National Hospital Organization Higashisaga Hospital
2)Research Institute of Medical Mass Spectrometry, Kurume University School of Medicine
Abstract
Background: Although long-term tubal ingestion, which causes carnitine deficiency, can be treated by intragastrical or intrajejunal L-carnitine administration, the biological effect of changing the administration route is unclear.
Objectives: To confirm carnitine deficiency risk in patients with severe motor and intellectual disabilities (SMIDs) and investigate the appropriate L-carnitine administration route.
Methods: Eighteen patients with SMIDs were enrolled. Nine patients each were nourished by tubal and oral ingestion. Tubal ingestion was via the intragastric and intrajejunal routes. Serum carnitine levels were measured by an enzyme cycling method from blood samples. In the tubal ingestion group, sampling was performed at several time points after L-carnitine (30 mg/kg) administration.
Results: Serum carnitine levels were lower than its normal range in the tubal ingestion group but remained within the normal range in the oral ingestion group. In 8/9 patients in the tubal ingestion group, levels reached the normal range by a single L-carnitine administration. There was no significant difference in the time-course change of levels between the intragastric and intrajejunal routes.
Conclusion: Patients with SMIDs receiving long-term tubal ingestion are at a high risk of carnitine deficiency. L-carnitine can be administered equally by intragastric and intrajeunal routes to patients with SMIDs.
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Case Report
Title
Hypoxic-ischemic Injury of the Brainstem in the Perinatal Period Can Be a Cause of Persistent Episodic Apnea and Dysphagia during Infancy: A Case Report
Author
Kei Iwata1) Shimpei Baba2) Atsuko Taki2) Kengo Moriyama2) Marie Ito1) Misaki Kodera1) Ryo Aoki2) Isaku Omori1) Mitsumasa Shimizu1) and Tomohiro Morio2)
1)Department of Neonatology, Tokyo Metropolitan Bokutoh Hospital
2)Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University
Abstract
We report the case of a 5-month-old girl with hypoxic-ischemic injury of the brainstem. The patient had a history of perinatal respiratory and circulatory failure. She exhibited persistent episodic apnea and dysphagia soon after birth. Her brainstem reflexes were decreased or absent. An electrophysiological examination revealed the disappearance of blink reflex. On magnetic resonance imaging (MRI), a T2-prolonged lesion was confirmed in the dorsal part of the caudal pons and medulla oblongata. Based on all these findings, the patient was diagnosed with hypoxic-ischemic injury of the brainstem, presumably caused by perinatal respiratory and circulatory failure. The dorsal brainstem represents a watershed area between the paramedian and long circumferential arteries. To date, there are few reports of neonates or infants with hypoxic-ischemic brain injury of the brainstem. This can be explained by the difficulty of establishing an exact diagnosis. We advocate that patients who have a history of perinatal respiratory and circulatory failure and exhibit symptoms implying brainstem impairment, such as apnea and dysphagia, should be evaluated using electrophysiological examinations and brain imaging focused on the brainstem, regardless of the severity of perinatal respiratory and circulatory failure.
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Case Report
Title
Successful Treatment of Catecholaminergic Polymorphic Ventricular Tachycardia with an Automated External Defibrillator Used by a Nursery School Worker
Author
Nozomi Tanabe1) Takahiro Nishihara1) Takafumi Obara1) Takahiro Yamashita1) Yoshifumi Miura1) Yuichiro Muto1) Nagisa Komatsu1) Katsuki Hirai1) Masahiro Migita1) and Koichi Yatsunami2)
1)Department of Pediatrics, Kumamoto Red Cross Hospital
2)Department of Pediatric Cardiology, Kumamoto City Hospital
Abstract
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia occurring in the absence of organic heart disease. The first symptom is often cardiac arrest. We encountered a 5-year-old boy with CPVT who suffered ventricular fibrillation while playing. A nursery school worker immediately initiated cardiopulmonary resuscitation and used the automated external defibrillator (AED) installed at the school. When emergency medical technicians arrived, sinus rhythm had been restored. Electrocardiography revealed bidirectional ventricular tachycardia and atrial fibrillation. CPVT was diagnosed. Propranolol was administered. However, supraventricular arrhythmias occurred. Flecainide was administered, resolving the supraventricular arrhythmias. On Day 22, he was discharged without neurological sequelae. A de novo mutation in the ryanodine receptor 2 (RyR2) gene was later confirmed. He had various supraventricular arrhythmias, but frequent supraventricular extrasystoles had been noted since early childhood. Psychomotor developmental delays had previously been pointed out, possibly in association with the RyR2 gene mutation.
AED installation at nursery schools and kindergartens is being left to the judgment of institutions. Although installing AEDs may vary among municipalities, there are gaps in AED availability rates between public and private institutions and between accredited and non-accredited institutions. Our experience highlights the desirability of AEDs being installed in all nursery schools and kindergartens.
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Case Report
Title
A Case of Fulminant Encephalopathy Secondary to Incompetent Kawasaki Disease
Author
Reiko Yamaguchi1) Yusaku Syudo1) Naoki Ando2) Yasushi Kanda1) and Mitsuji Iwasa1)
1)Department of Pediatrics, Nagoya Daini Red Cross Hospital
2)Josai Kids Clinic
Abstract
Kawasaki disease is associated with various complications, but severe encephalopathy is very rare. A 3-year-old girl was admitted to our hospital on the 3rd day of illness with fever and swelling of the right cervical lymph node. We suspected posterior pharyngeal abscess on the basis of contrast-enhanced computed tomography findings. We continued medical treatment, and the symptoms improved. On the 13th day of illness, fever reappeared, confirming the four main symptoms of Kawasaki disease (bilateral nonexudative conjunctivitis, rash, extremity changes, and cervical lymphadenopathy). Convulsions appeared on the 16th day of illness. A head MRI showed a diffuse, high-signal area in the brainstem, bilateral thalamus, and caudate nucleus, as well as white matter on T2-weighted images and FLAIR. We made a diagnosis of acute encephalopathy and promptly initiated treatment; however, exacerbation of cerebral edema and brainstem swelling occurred 12 h later. At this time, the encephalopathy was believed to mainly comprise angiogenic edema; however, the symptoms developed rapidly and exhibited extremely serious irreversible cytotoxicity within 12 h of onset. The clinical course and image findings are both nonspecific and considered valuable and are, therefore, reported herein.
Encephalopathy associated with Kawasaki disease is very rare, but certain cases, such as this one, follow a very serious course.
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Case Report
Title
A Case of an Infant Suspected of Primary Antiphospholipid Syndrome Distinguished from Abuse
Author
Ryo Matsuoka1)3) Takahiro Aoki2) Satoru Ishikawa1)3) Tomoko Hara1) Ryusuke Nambu1) Shin-ichiro Hagiwara1) Katsuyoshi Ko2) Seiichi Kagimoto1) and Hiroyuki Ida3)
1)Division of General Pediatrics, Saitama Children's Medical Center
2)Division of Hematology/Oncology, Saitama Children's Medical Center
3)Department of Pediatrics, The Jikei University School of Medicine
Abstract
In children, antiphospholipid syndrome (APS) is sometimes associated with infection and causes bleeding or thrombus symptoms. We present a case of a 1-year-old male patient with APS who was initially suspected of being abused.
The patient had purpura repeatedly and subsequent episodes of human metapneumovirus (hMPV) infections. His blood exam was normal (clotting function, only PT and APTT were evaluated at this time). He was suspected being abused because of repeated purpura of unknown origin. After admission, he showed FDP 1,012 μg/dL and D-dimer 533 ng/dL in the initial blood samples. Additionally, anticardiolipin antibodies (aCL) IgG and anti-phosphatidylserine-prothrombin (aPS/PT) antibodies levels were elevated. Purpura improved promptly and FDP and D-dimer normalized within a month. aPS/PT antibody values were negative within 7 months of the initial levels and other symptoms such as SLE were not observed. After 2 years, aCL-IgG remained positive.
This case report suggests that APS associated with infection must be differentiated from suspected abuse. In children, thrombus symptoms of APS are rare, and thus it is important to evaluate thrombotic pathologies.
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Case Report
Title
Recurrent Stevens-Johnson Syndrome Thought to Be Associated with Mycoplasma pneumoniae and an Antipyretic Drug
Author
Yukiko Hirota Kaori Nakatani Kio Tanaka Ryuichi Nakagawa and Koji Kiyohara
Department of Pediatrics, Tokyo-Kita Medical Center
Abstract
A 13-year-old boy presented to the hospital complaining of a conjunctival injection, cold sores on the lips and inside the mouth. The patient developed fever 4 days prior to the administration and next day developed a cough, so he took "cold medicine" containing acetaminophen. One day prior to the administration, the patient vomited and had diarrhea and took acetaminophen twice. He had a history of same episode with fever, oral lesions, and swollen eyelids three years prior to this event, and was given a diagnosis of Stevens-Johnson syndrome (SJS) and recovered with treatment of steroids. Drug-induced lymphocyte stimulation test (DLST) for acetaminophen was suspected positive at the first episode. SJS was diagnosed based on his clinical conditions and the previous episode, and we administered prednisolone (PSL) 1.5 mg/kg/day intravenously. On day two skin rash and genital erosions, and on day four pseudomembranous on conjunctivas were appeared. His oral lesions were severe so he required parenteral nutrition. On day five he was afebrile, and oral lesions eventually resolved. We gradually tapered PSL and he was discharged on day 28 with 0.5 mg/kg/day of oral PSL. Mycoplasma pneumoniae antibodies (particle agglutination method) increased from 160 fold to 1,280 fold and DLST for acetaminophen was positive. We suspected that he had a recurrent SJS associated with M. pneumoniae infection and acetaminophen.
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