Acta Paediatrica Taiwanica

賴鈺嘉

Táiwān érkē yī xuéhuì. Xuéhuì zázhì/Acta paediatrica taiwanica

台灣兒科醫學會第163屆學術演講會教育演講：證據醫學與小兒科醫學－證據醫學基礎及Cochrane中心之介紹

台湾儿科医学会第163届学术演讲会教育演讲：循证医学与儿科医学——循证医学基础及Cochrane中心介绍

李 建興

H-Type Tracheoesophageal Fistula: Report of One Case

H型气管食管瘘：一例报告

呂 政建

Positive Blood Cultures in Pediatric Emergency Department Patients: Epidemiological and Clinical Characteristics

儿科急诊患者血培养阳性病例的流行病学及临床特征

儿科急诊患者阳性血培养：流行病学和临床特征

Inflammatory myofibroblastic tumor (IMT) is a very rare benign tumor composed of myofibroblastic spindle cells of uncertain etiology, which can occur at any age and affect any organ system. More and more cases of IMT in children have been described in pediatric literature in recent years. However, this tumor occurring intraabdominally in children has rarely been reported in Taiwan. Here we present a 1-year-9-month-old boy who had fever and abdominal pain only for 2 days, symptoms mimicking acute abdomen. After imaging study, a huge tumor nearly 10 cm in diameter was incidentally found over the right abdomen with unknown origin and nature. After surgical removal of the tumor, IMT was confirmed by the pathological findings. It is very difficult to make an accurate preoperative diagnosis on this tumor according to past experience, so the role of pathological diagnosis with immunohistochemical study becomes important. This case illustrates that IMT should be considered as a possible cause of intra-abdominal mass in children who have fever of unknown origin.

Hsiu-Huei Wen

Kui-Lin Cheng

Yau-Kan Hung

Pei-Yeh Chang

Inflammatory myofibroblastic tumor presenting as acute abdomen: report of one case.

表现为急腹症的炎性肌纤维母细胞瘤：一例报告。

Cri-du-chat syndrome is a genetic disorder associated with various sized deletions of the short arm of chromosome 5. There are typical physical features, but individual phenotypes vary considerably.The records of 23 patients with cri-du-chat syndrome admitted to Mackay Memorial Hospital from June 1984 to February 2006 were retrospectively reviewed. Data abstracted from the records included abnormal facial features and physical findings, results of echocardiography, bronchoscopy, auditory evoked potential, visual evoked potential, brain ultrasonography, and karyotype. We examined the various clinical phenotypes to see if there was an association with specific karyotypes.Among congenital heart conditions, atrial septal defect (8/15, 53%) was the most common, followed by ventricular septal defect (4/15, 26%), tricuspid regurgitation (4/15, 26%) and patent ductus arteriosus (3/15, 20%). Laryngomalacia was the most frequent airway problem (8/23, 34%) and strabismus the commonest visual disorder (1/23). A high percentage of patients had impaired hearing (5/23, 21%). There was no clear relationship found between deletion size and major clinical manifestations in this study.Karyotype is not a reliable indicator of specific organ involvement in cri-du-chat syndrome. However, karyotyping is still useful, particularly if parental translocation are found to be involved. It helps for prenatal diagnosis of next pregnancy.

Chia-Ying Chang

Shuan-Pei Lin

Hsiang-Yu Lin

Yen-Jiun Chen

Hsin-An Kao

Chun-Yan Yeung

Chyong-Hsin Hsu

Hsin Chi

Cri-du-chat syndrome.

猫叫综合征。

Harlequin ichthyosis (HI) is the most devastating form of skin disorder, which is inherited as autosomal recessive trait related to consanguineous marriage. Although prenatal examination has become scheduled and convenient throughout Taiwan, an unexpected case of HI in a male premature infant born at 32 weeks of gestation was presented. The parents were healthy, neither relatives nor having history of congenital abnormality. We report our management and the massive impact left on both parents. We believe this is an extremely rare case in Taiwan.

Peck-Foong Thien

Wen-Jue Soong

Ping-Yao Wang

Ting-Rong Hsu

Harlequin ichthyosis: report of one case.

大疱性鱼鳞病样红皮病：一例报告。

A. The prevalent pathogen is different in different agegroups.B. Atypical pneumonia1.Viral infections prevail in children younger than5 years of age, especially respiratory syncytialvirus. Other viral etiologies include influenzavirus, parainfluenza virus, adenovirus, humanmetapneumovirus, rhinovirus, and cytomegalovirus.2.

Ping-Ing Lee

Cheng-Hsun Chiu

Po-Yen Chen

Chin-Yun Lee

Tzou-Yien Lin

Guidelines for the management of community-acquired pneumonia in children.

儿童社区获得性肺炎管理指南。

The close relationship between thyroid disorder and Down syndrome (DS) had been widely reported in the literature. The aim of this study was to assess the prevalence rate and the pattern of thyroid dysfunction in DS in Taiwan.A total of 50 Down syndrome (DS) patients from Yang-Ming Home for Disabled, were recruited. In all patients, serum T3, T4, free T4, TSH, thyroid peroxidase antibody (anti-TPO) and anti-TSH receptor antibody were measured. The control group consisted of 212 healthy adults who underwent routine health examination in this hospital.Among 50 DS patients, 8 had subclinical hypothyroidism and one had hyperthyroidism. There was no statistically significant in gender and age between DS and control groups, however, TSH anomaly was significantly higher in the DS group (p = 0.008). Regarding thyroid autoantibodies, anti-TPO was positive in 13 cases (26%) and anti-TSH receptor was positive in 2 cases (4%). Statistically, there was no significant difference in TSH anomaly (p = 0.7), positive anti-TPO antibody (p = 0.097) and positive anti-TSH receptor antibody (p = 1.0) between males and females. The increased TSH levels had no statistically positive correlation with anti-TPO (p = 0.386) or anti-TSH receptor antibody (p=1) in this study. The prevalence of thyroid dysfunction was 18% (9/50) in DS patients in Taiwan. Most of them showed subclinical compensated hypothyroidism (16%, 8/50), and one (2%, 1/50) showed hyperthyroidism.Thyroid dysfunction is common in DS patients, so periodic thyroid function tests should be performed and early treatment should be given to prevent further intellectual deterioration and improve overall development.

Publication Information	Publisher: Taiwan Pediatric Association，Publishing cycle: ，Journal Type: journal，Open Access Journals: No
Basic data	Year of publication: 1999，Proportion of original research papers: ，Self Citation Rate:， Gold OA Rate:

Acta Paediatrica Taiwanica

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台灣兒科醫學會第163屆學術演講會教育演講：證據醫學與小兒科醫學－證據醫學基礎及Cochrane中心之介紹

H-Type Tracheoesophageal Fistula: Report of One Case

Positive Blood Cultures in Pediatric Emergency Department Patients: Epidemiological and Clinical Characteristics

H-Type Tracheoesophageal Fistula: Report of One Case

Positive Blood Cultures in Pediatric Emergency Department Patients: Epidemiological and Clinical Characteristics

Inflammatory myofibroblastic tumor presenting as acute abdomen: report of one case.

Cri-du-chat syndrome.

Harlequin ichthyosis: report of one case.

Guidelines for the management of community-acquired pneumonia in children.

Thyroid dysfunction in patients with Down syndrome.