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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.116, No.9, September 2012
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Original Article
Title
Efficacy of Early Corticosteroid Therapy in the Treatment of Refractory Mycoplasma pneumoniae Pneumonia
Author
Akihiro Kataoka Dai Asada Jiro Takeuchi Daisuke Jonen Mioko Mori Yasuhiro Mizushima and Yoshihiro Wakazono
Department of Pediatrics, Kyoto Katsura Hospital
Abstract
Objective: This study aimed at evaluating the response to macrolide treatment and efficacy of early steroid therapy in children with Mycoplasma pneumonia.
Methods: Between January 2006 and June 2011, 40 children with Mycoplasma pneumonia were hospitalized, of which 13 with serious symptoms were administered steroids. Effectiveness of steroids and the response to macrolides were evaluated on the basis of the clinical symptoms, including body temperature and respiratory conditions.
Results: After treatment, the proportion of patients showing a good response to macrolide treatment decreased and that requiring steroid treatment increased. A prompt improvement in clinical symptoms was observed in all patients treated early with steroids. Systemic corticosteroid therapy during the early stage of the disease was associated with rapid remission of high fever and no deterioration in respiratory conditions, including pleural effusion, in all patients. A total of 8 patients who did not receive early steroid therapy required the therapy later because of the development of complications such as pleural effusion and meningitis. The severity of Mycoplasma infection depends on excessive immune responses rather than direct invasion; therefore, early steroidal intervention is recommended for the treatment of Mycoplasma pneumonia.
Conclusion: Mycoplasma pneumonia has been showing an increasing trend of resistance to macrolides. Patients with elevated levels of lactate dehydrogenase can be candidates for steroid therapy. We found that early administration of corticosteroids is an effective alternative therapy for patients showing a poor response to macrolide treatment.
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Original Article
Title
The Experience of Propranolol Treatment for Infantile Hemangiomas and Kasabach-Merritt Syndrome
Author
Ryosuke Matsuno1) Katsuyoshi Koh1) Ayumu Arakawa1) Masashi Seki1) Hiroyoshi Takahashi1) Yoshihiro Gocho1) Motohiro Kato1) Yoshihisa Nagatoshi1) Kotaro Hine2) Masaki Simizu2) Itaru Iwama3) Seichi Kagimoto3) and Ryoji Hanada1)
1)Department of Hematology/Oncology, Saitama Children's Medical Center
2)Department of Neonatology, Saitama Children's Medical Center
3)Department of General Pediatrics, Saitama Children's Medical Center
Abstract
Some of infantile hemangiomas (IHs) are proliferating from a few months to a few years which can cause function- or life-threatening risks. Dramatic improvement of IHs to oral propranorol was recently reported. Here we report a treatment with propranorol for 9 patients of IHs and 2 patients of Kasabach-Merritt syndrome (KMS) in our center.
Patients: Nine patients of IHs and two patients of KMS were treated with propranorol, including 5 patients of IHs already treated with steroid or laser. After confirmation of normal function for echocardiographic evaluation, we started administration of oral propranolol. The evaluation of effectiveness was the change of tumor size in the surface and size of magnetic resonance imaging (MRI) in the body.
Results: Prompt regression was seen in all cases in IHs. There were no adverse events, such as low blood pressure, low heart rates, or hypoglycemia. There was no effect in two patients of KMS.
Conclusion: Propranorol is effective and safe for IHs. Propranorol can become a first-line treatment for IHs with function- or life-threatening risk.
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Original Article
Title
Clinical Features in 26 Patients with CHARGE Syndrome
Author
Aki Ishikawa1) Keisuke Enomoto1) Noritaka Furuya1) Kouji Muroya2) Yumi Asakura2) Masanori Adachi2) and Kenji Kurosawa1)
1)Division of Medical Genetics, Kanagawa Children's Medical Center
2)Division of Endocrinology & Metabolism, Kanagawa Children's Medical Center
Abstract
CHARGE syndrome is a congenital malformation syndrome characterized by a nonrandom occurrence of anomalies including coloboma, heart defects, choanal atresia, retarded growth and development, genital abnormalities, and ear anomalies/deafness. To elucidate the natural history and medical managements of the syndrome, we reviewed the clinical features of 26 patients with CHARGE syndrome who received follow-up care at our Children's Medical Center. The ages at diagnosis varied widely from neonates to adolescents. The most common clinical findings, including external ear malformations, hearing loss, and developmental delays, were recognized in all patients, followed by growth retardation (92%), coloboma (77%), congenital heart defects (84%), and genital hypoplasia/hypogonadotropic hypogonadism (84%). Eight patients (30%) had choanal atresia as a cardinal feature of CHARGE syndrome, but esophageal atresia, which is one of the major complications, was found in only one case. An MRI survey on the semicircular ducts and olfactory bulb provided useful information for clinical diagnosis of the syndrome. Because of cognitive retardation associated with visual disturbance and deafness, evaluation of the patients' potentials for development is difficult. However, approximately 80% of patients were able to communicate by manualism, gestures, and signs, and these results suggested a discrepancy between congenital potential ability and their real performance. Patients with CHARGE syndrome frequently had behavioral difficulties, attention deficits, and hyperactivity. These characteristic types of behavior may be partially related to problems of arousal, self-regulation, and frustration. Ophthalmologic evaluation and auditory examination should be performed as early as possible in CHARGE syndrome patients. Our study results will be useful for diagnosis, medical managements of complications, and rehabilitations of CHARGE syndrome patients. We believe that a better knowledge of the underlying syndrome leads to better familial understanding and acceptance, and could provide insight into the prognosis.
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Original Article
Title
Kawasaki Disease in 3 Infants under 60-days Old
Author
Daisuke Yanagisawa Koichiro Saito Daigo Ito Akihiko Yajima and Naoi Fukasawa
Department of Pediatrics, Maebashi Kyoritsu Hospital
Abstract
The incidence of Kawasaki disease (KD) in infants, particulary in infants under 60-days old is low. We encountered 3 cases of Kawasaki disease (KD) in infants under 60-days old. All showed fever or exanthema in the early stages of the illness, and the clinical course was showed typical clinical manifestations in all 3 cases. They also responded well to aspirin and gamma-globulin treatment and had with no cardiac complications. However, KD in early infancy usually has characteristics of incomplete KD and relatively high frequency of cardiac complications. Therefore, we should consider the differential diagnosis of KD, if infants present with fever and exanthema during early infancy, because exanthema is a clear indication of KD from other febrile exanthema-related diseases because of the high frequency of exanthema even in this age group. In addition, it is important to perform echocardiography for early diagnosis and treatment when KD is suspected because of the posibility of incomplete manifestations and the high risk of cardiac complications.
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Original Article
Title
Clinical Features of Spontaneous Pneumomediastinum
Author
Mariko Abe1) Takeshi Taketani1)2) Seiji Fukuda1) Kenji Yasuda1) Takeshi Kasai3) and Seiji Yamaguchi1)
1)Department of Pediatrics, Shimane University Faculty of Medicine
2)Division of Blood Transfusion, Shimane University Hospital
3)Department of Pediatrics, Unnan Municipal General Hospital
Abstract
Spontaneous pneumomediastinum (SPM) is a rare disease with the symptom of sudden chest pain in healthy individuals. Patients visit different clinical divisions; however, they may be seen in the pediatric outpatient clinic. Herein, we present the clinical features of 11 cases of SPM treated at our hospital and our affiliated clinics. All patients were teenage boys, with the exception of one girl. The median body mass index was 18.2. Seven of them experienced symptoms associated with elevated airway pressure, 9 complained of chest pain or discomfort, and one had breathing difficulties. One complained of an unusual sore throat and swallowing pain, which was distinct from that usually experienced with a common cold, without chest symptoms. None of them showed the classical three symptoms, i.e., simultaneous chest pain, neck pain and dysphasia. The diagnosis of all subjects was made by the presence of air leakage in the mediastinum on chest X-ray or CT scan. Some patients were hospitalized for intravenous antibiotic injection and all recovered without any complications. SPM should be considered in the diagnosis of unusual sore throat or swallowing pain, especially in thin adolescent boys. Our study suggests that antibiotic administration or hospitalization is not necessary for patients with SPM who do not show any signs of vomiting, progressive dyspnea or risk for mediastinitis resulting from spontaneous rupture of the esophagus.
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Original Article
Title
A Case of Racemose Hemangioma with Recurrent Hemoptysis
Author
Sayaka Ishikawa Tetsujiro Shirahashi Yosuke Imanishi Munetoshi Nanbu Nobuyuki Doichi Yuuya Nakada Eri Shinozaki Hiroyuki Kitano Natsuhito Nishio Yasuhisa Ueno Seiki Horita and Minoru Kubo
Ishikawa Prefectural Central Hospital, Ishikawa Medical Center for Maternal and Child Health, Pediatrics
Abstract
Racemose hemangioma of the bronchial artery is characterized by dilatation, tortuosity, or flexion of the bronchial artery. In some cases, the bronchial artery may establish an abnormal communication with a bronchial vein or the pulmonary artery. This condition is rare, and there are very few reports of racemose hemangioma in children.
We describe a 7-year-old boy in whom a definitive diagnosis had not been established despite multiple past episodes of hemoptysis. He vomited blood twice during the daytime and was admitted to our hospital the same day. Computed tomography (CT) examination of the lungs revealed a pulmonary arteriovenous malformation (AVM). However, there was no evidence of AVM on pulmonary angiography; which instead showed, several racemose hemangiomas. The hemangiomas formed a shunt from the bronchial to the pulmonary artery. Thus, we performed embolization of the right middle bronchial artery, which showed the most extensive involvement.
We speculated that the recurrent hemoptysis was attributable to the racemose hemangiomas detected by pulmonary angiography. There are several treatment options including bronchial artery embolization using sponges or coils, bronchial artery ligation, and pulmonary lobectomy for this condition.
However, all of these methods have advantages and disadvantages. Hence, no definitive treatment has yet been established. Although racemose hemangioma is a rare condition, when accompanied by serious hemoptysis, it may result in an emergent situation. Therefore, patients with this condition require careful follow up.
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