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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.118, No.8, August 2014
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Original Article
Title
Five Cases of Childhood Pneumonia Caused by Chlamydophila pneumoniae Microbiologically Diagnosed by DNA Detection Using the Loop-mediated Isothermal Amplification Method
Author
Naoko Nishimura Takao Ozaki Kensei Gotoh Suguru Takeuchi Fumihiko Hattori Kazuhiro Horiba Mai Isaji Yu Okai Haruki Hosono and Koji Takemoto
Department of Pediatrics, Konan Kosei Hospital
Abstract
Chlamydophila pneumoniae is a pathogen known to cause childhood community-acquired pneumonia, but there are few tests that can diagnose this disease in the acute period and its actual prevalence among children in Japan are not well understood. In this study, 1,134 cases of pediatric pneumonia from our department during a 29 month period from April 2009 to August 2011 were investigated, and identification of C. pneumoniae DNA by loop-mediated isothermal amplification (LAMP) was attempted. Five cases (0.4%) were DNA positive, and the microbiologically diagnosis was C. pneumoniae pneumonia. An increase of C. pneumoniae IgM antibodies (index≥ 2.00) in 4 cases and seroconversion of C. pneumoniae IgM antibody in one case were observed in paired sera (acute and recovery periods). Moreover, co-infection with Mycoplasma pneumoniae was confirmed using the LAMP method in two out of five cases. The patients were aged 4-13 years, and all had fever higher than 38.5°C. Three cases had a CRP≥ 2.0 mg/dl, and one had a WBC≥ 10,000/μl. Only one case each showed prolonged coughing and wheezing, which are characteristics of C. pneumoniae pneumonia, so identification by clinical symptoms was difficult. All cases improved with clarithromycin, and no complications were observed. The LAMP method can rapidly make a microbiologocally diagnosis and is considered useful in the treatment of C. pneumoniae pneumonia.
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Original Article
Title
Changes of Carnitine and Biochemical Variables after Levocarnitine Administration in Patients with Severe Motor Intellectual Disabilities
Author
Yoko Takeda1) Kiyotaka Tomiwa1) Hajime Kin1) Chiharu Kawaguchi1) Yukie Higashiyama2) Ayako Nagai2) and Masaru Kubota2)
1)Department of Pediatrics, Todaiji Medical and Education Center
2)Faculty of Human Life and Environment, Nara Women's University
Abstract
[Objective] The prevalence of carnitine deficiency is high in patients with severe motor intellectual disabilities. The possible causative factors include enteral tube feeding, administration of anti-convulsants, and low body storage of carnitine presumably due to decreased muscle volume. The purpose of the present study was to determine the effect of levocarnitine by comparing carnitine and various biochemical variables before and after administration of levocarnitine.
[Methods and Patients] Twenty-six patients with severe motor intellectual disabilities were given 30 mg/kg/day of levocarnitine for 2 weeks. In 20 patients at risk for the recurrence of carnitine deficiency after cessation of levocarnitine, the same dose was administered for a further 10 months. Serum levels of carnitine and various biochemical variables were determined before and after administration.
[Results] Initial serum total-, free-, and acyl- carnitine levels were significantly lower than the reference values. They were almost normalized after 2 weeks of levocarnitine administration. Uric acid and amino-terminal pro-brain natriuretic peptide levels were also significantly improved. Although the overall decrease of ammonia level was not significant, those in 8 out of 9 cases with higher levels initially fell within the normal range. After 10 months of administration, carnitine levels did not change significantly in comparison with those after 2 weeks of administration. Uric acid and choline esterase levels were found to be significantly lower than those after 2 weeks administration.
[Conclusion] Levocarnitinine (30 mg/kg/day) administration restored the carnitine levels in patients with severe motor intellectual disabilities having carnitine deficiency. The fact that the restoration of carnitine was accompanied by improvement of several biochemical variables verified the effectiveness of levocarnitine administration.
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Original Article
Title
Clinical Characteristics of Infants Who Experienced Apparent Life-threatening Events
Author
Riyo Ueda1) Takanobu Maekawa1) Osamu Nomura1) Akira Ishiguro1) Hirokazu Sakai1) and Satoshi Nakagawa2)
1)Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development
2)Department of Critical Care and Anesthesia, National Center for Child Health and Development
Abstract
Epidemiology and clinical characteristics of apparent life-threatening events (ALTE) have not been thoroughly investigated in Japan. We conducted a retrospective review of medical records of ALTE cases at the National Center for Child Health and Development (NCCHD) to clarify the clinical characteristics of ALTE. We examined infant cases (less than 1 year old) of ALTE who visited the Emergency Room of NCCHD during the period from March 2002 to January 2012. A total of 112 infants, including 55 males and 57 females, were found to have ALTE in the study period. The age at the event varied from 0 to 49 weeks, with a median of 7 weeks. The number of prematurely born infants (≤36weeks at birth) was 9 (8%) and that of infants having underlying diseases was 16 (14%). Symptoms of ALTE include pallor (79%), muscular hypotonia (43%) and breathing difficulties (32%). As the diagnostic investigations of ALTE, complete leukocyte counts, blood gas analysis, serum chemistry, blood culture, electrocardiogram and chest X-ray were performed in almost all infants. The most frequent diagnosis was gastro-esophageal reflux (27%). 19 cases (17%) presented recurrent events during the hospitalization and 5 cases (4%) had recurrent events after more than 1 month from the first event. We followed up 79 cases for 6 months after the first event, among which there was 1 fatal case. We recommend close observation for infants who have recurrent events during hospitalization. Further researchvis needed to clarify the long-term prognosis of ALTE patients.
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Case Report
Title
A Pediatric Case of Basedow Disease Diagnosed Based on an Episode of Hypokalemic Periodic Paralysis
Author
Yoichi Iki Yoshie Nakamura Masatoshi Nakata Hiroshi Mizumoto Takakazu Yoshioka Mitsutaka Shiota Atsuko Hata Ken Watanabe and Daisuke Hata
Department of Pediatrics, Tazuke Kofukai Kitano Foundation, Medical Research Institute, Kitano Hospital
Abstract
This report describes a pediatric case of Basedow disease diagnosed based on an episode of hypokalemic periodic paralysis. A 15-year-old boy was admitted because of sudden gait inability one morning. He had non-tender enlargement of thyroid and muscle weakness of the extremities. Laboratory examinations revealed hypokalemia (2.0 mEq/L), and first-degree atrioventricular block and prolonged QT (QTc 0.54 s) on electrocardiogram, confirming a diagnosis of hypokalemic periodic paralysis. Six hours after starting intravenous infusion of potassium, muscle weakness and electrocardiographic abnormality had improved with normalization of serum potassium (4.2 mEq/L). Further examinations revealed hyperthyroidism (thyroid-stimulating hormone< 0.01 μIU/mL, free thyroxine 4.27 ng/dL, and free thyronine> 30.0 pg/mL) with a high titer of serum anti-thyroid-stimulating hormone receptor antibody (185 IU/L). Thyroid ultrasound detected diffuse enlargement of the thyroid gland with increased blood flow. He was given a definitive diagnosis of Basedow disease with thyrotoxic periodic paralysis (TPP). Treatment with thiamazole and propranolol were started respectively on the second and the fourth day after admission. He became able to walk at 10 hr after initiation of therapy. Administration of potassium was discontinued on the fourth day after admission. The next day, however, he again became unable to walk independently with hypokalemia (3.0 mEq/L) at night, which was probably induced by eating a huge lunch. Oral administration of potassium improved thy muscle weakness in the next morning. After progressing well thereafter, he was discharged on the ninth day of administration.
The report of pediatric TPP is rare because of the high frequency of familial periodic paralysis as a main cause of hypokalemic periodic paralysis. Consideration of TPP in the differential diagnosis of pediatric muscle weakness is important because normalization of thyroid function is necessary to prevent exacerbation of TPP, and fatal arrhythmia can occur in the acute stage of TPP.
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Case Report
Title
A Case of Obturator Internus and Externus Pyomyositis with Bacteremia
Author
Masaya Suematsu1) Mihoko Yamaguchi1) Satoshi Sakaue1) Yoshiki Katsumi1) Osamu Otabe1) Hisato Ito1) and Fumihiro Fujiwara2)
1)Department of Pediatrics, Nantan General Hospital
2)Fujiwara Pediatric Clinic
Abstract
Pyomyositis is a subacute, deep bacterial infection of skeletal muscle. Although pyomyositis is a rare disease, early accurate diagnosis and treatment are necessary for a good outcome in adjacent joints and full physical recovery. Here, we report the case of a 9-year-old boy with obturator pyomyositis. He fell from a bicycle and grazed his left knee. Two weeks later, he suddenly had left hip pain and high fever. The next day, he was transferred to our hospital for scrutiny. He could not walk because of severe hip pain, and had oppressive pain in the left inguinal region. Laboratory examinations revealed a white blood cell count of 11,810/μL and a C-reactive protein level of 5.2 mg/dL. A magnetic resonance imaging (MRI) scan showed high intensity on the T2-weighted image and T2-weighted short-tau inversion recovery in the left obturator internus and externus muscles, suggesting a diagnosis of left obturator pyomyositis. Staphylococcus aureus was detected in the blood culture. The patient was given a two-week course of intravenous cefazolin, and was discharged on the 15th day after admission with oral cefdinir treatment for the following two weeks, for a total of four weeks of treatment.
As the patient belongs to a futsal club, his obturator was likely damaged because of the futsal exercises. We therefore believe that the infection spread hematogenously from a graze on his left knee to his left obturator, which was already damaged.
Therefore, in patients presenting with hip pain and high fever, a diagnosis of obturator pyomyositis should be considered. Early diagnosis with an MRI scan can enable successful treatment.
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Case Report
Title
Clinical Courses of Five Patients with Various "Lethal" Skeletal Dysplasias
Author
Toshio Okamoto1) Yumi Kawata2) Hiroko Asai1) Etsushi Tsuchida1) Fumikatsu Nohara1) Hiroyuki Kitamura3) Yoshiaki Sasaki3) Ken Nagaya1) and Hiroshi Azuma2)
1)Department of Neonatology, Perinatal Center, Asahikawa Medical University Hospital
2)Department of Pediatrics, Asahikawa Medical University
3)Department of Pediatrics, Abashiri Kosei Hospital
Abstract
We report 5 patients with "lethal" skeletal dysplasia (campomelic dysplasia, platyspondylic lethal skeletal dysplasia Torrance type, dyssegmental dysplasia Rolland-Desbuquois type, otopalatodigital syndrome type II, and short-rib polydactyly syndrome type III). Fetal computed tomography, performed on 3 patients, were useful in evaluating pulmonary hypoplasia and presenting accurate information regarding the parents. Initial resuscitation was performed on all patients, 4 of whom survived 1 year through appropriate respiratory support and 1 had withdrawal of intensive treatment because of severe pulmonary hypoplasia. All survivors underwent tracheostomy, 3 of whom could be discharged home, including 1 receiving mechanical ventilation. All survivors showed respiratory failure and other complications including scoliosis, hydronephrosis, cataract, and hearing impairment. Given appropriate intensive treatment especially respiratory support, considerable numbers of patients with severe skeletal dysplasia traditionally stigmatized as "lethal" had long-term survival, albeit with slow psychomotor development. Careful pre-and-postnatal counseling with parents based on accurate information especially about pulmonary hypoplasia is necessary for planning individualized management on these patients.
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