• Clinical and Experimental Pediatrics
• /
• 제63권3호
• /
• pp.79-87
• /
• 2020

Inherited platelet disorders (IPDs), which manifest as primary hemostasis defects, often underlie abnormal bleeding and a family history of thrombocytopenia, bone marrow failure, hematologic malignancies, undefined mucocutaneous bleeding disorder, or congenital bony defects. Wide heterogeneity in IPD types with regard to the presence or absence of thrombocytopenia, platelet dysfunction, bone marrow failure, and dysmegakaryopoiesis is observed in patients. The individual processes involved in platelet production and hemostasis are genetically controlled; to date, mutations of more than 50 genes involved in various platelet biogenesis steps have been implicated in IPDs. Representative IPDs resulting from defects in specific pathways, such as thrombopoietin/MPL signaling; transcriptional regulation; granule formation, trafficking, and secretion; proplatelet formation; cytoskeleton regulation; and transmembrane glycoprotein signaling are reviewed, and the underlying gene mutations are discussed based on the National Center for Biotechnology Information database and Online Mendelian Inheritance in Man accession number. Further, the status and prevalence of genetically confirmed IPDs in Korea are explored based on searches of the PubMed and KoreaMed databases. IPDs are congenital bleeding disorders that can be dangerous due to unexpected bleeding and require genetic counseling for family members and descendants. Therefore, the pediatrician should be suspicious and aware of IPDs and perform the appropriate tests if the patient has unexpected bleeding. However, all IPDs are extremely rare; thus, the domestic incidences of IPDs are unclear and their diagnosis is difficult. Diagnostic confirmation or differential diagnoses of IPDs are challenging, time-consuming, and expensive, and patients are frequently misdiagnosed. Comprehensive molecular characterization and classification of these disorders should enable accurate and precise diagnosis and facilitate improved patient management.

• 대한소아치과학회지
• /
• 제37권4호
• /
• pp.545-550
• /
• 2010

Freeman-Sheldon Syndrome(FSS)은 드물게 발생하는 선천성 유전 질환으로 휘파람을 부는 듯한 특징적인 안모로 인해 'Whistling face syndrome'으로 불리워진다. 또한 편평한 안모, 긴 인중(philtrum), 낮은 비연골이 나타나 두드러진 안면이상을 보이고, 내반족(club foot, 內反足), 손가락의 관절구축(joint contracture)으로 인한 풍차 모양의 손을 가지며, 지능은 보통 정상이다. 본원에 내원한 환아는 이 질환의 특징적 양상인 구강 주위 근육의 수축으로 소구증 및 개구제한, 높은 구개 및 부정교합, 치열의 심한 총생을 보여 구강위생이 매우 불량하였고 치과치료에 비협조적이었다. 소아치과적 행동조절 및 구강위생관리, 섭식장애의 상담으로 치과적 문제는 다소 개선되었으나, 안과 및 정형외과, 신경정신과, 교정과, 마취과적 문제로 인해 다양한 협진이 필요하며 계속적인 관찰 및 치료가 요구되기에 이를 보고하는 바이다.

• Journal of Korean Neurosurgical Society
• /
• 제52권4호
• /
• pp.417-419
• /
• 2012

Tumors of the central nervous system are common in the pediatric population and constitute the second most prevalent tumor type in children. Within this group, spinal cord tumors are relatively rare and account for 1 to 10% of all pediatric central nervous system tumors. We describe a very rare case of an intradural extramedullary spinal cord tumor with a subcutaneous mass and discuss its clinical presentation, pathogenesis, and treatment. A male infant was delivered normally, with uneventful development. At 16 days post-delivery, his family took him to a pediatrician because of a mass on his upper back. Magnetic resonance imaging of the thoracic spine revealed a well-demarcated soft-tissue mass with central cystic change or necrosis at the subcutaneous layer of the posterior back (T2-7 level). Another mass was found with a fat component at the spinal canal of the T1-3 level, which was intradural extramedullary space. After six weeks, the spinal cord tumor and subcutaneous mass were grossly total resected; pathologic findings indicated an atypical myxoid spindle cell neoplasm, possibly nerve sheath in type. The final diagnosis of the mass was an atypical myxoid spindle cell neoplasm. The postoperative course was uneventful, and the patient was discharged after nine days without any neurological deficit. We report a rare case of an intradural extramedullary spinal tumor with subcutaneous mass in a neonate. It is necessary to monitor the patient's status by examining consecutive radiologic images, and the symptoms and neurological changes should be observed strictly during long-term follow-up.

• 여성건강간호학회지
• /
• 제6권4호
• /
• pp.516-527
• /
• 2000

The purpose of this study was to investigate belief and attitudes on breastfeeding of physicians and nurses. A questionnaire was mailed to obstetricians, pediatricians and nurses at the department of pediatrics or obstetrics of university hospital and private hospital in Seoul and Choong-Chung Province) in South Korea. Total numbers of study subjects were 346 (pediatrician 67, obstetrician 41, nurse 238). The results were as follows : 1. The main reasons not to educate breast feeding in the hospitals were a lack of interest of the health professionals, a lack of educators and education programs. 2. The respondents thought that the best ways for lactating mother to get advices about breastfeeding during the first month were to give a call to health professionals in the hospitals where she had been delivered, or call to relatives or friends. 3. Breastfeeding attitudes of physicians and nurses did not differ according to gender, job, or type of the hospitals they work. 4. Breastfeeding attitudes were related with personal breastfeeding experience, breastfeeding knowledge, extracurricular education experience, encouragement experience. Physicians and nurses should give appropriate advices and support to lactating mothers to increase breastfeeding rate. They were, however, ill-prepared to counsel breastfeeding mothers. Therefore, it is necessary to instruct breastfeeding in the curriculum of the medical and nursing schools, and incorporate clinically based breastfeeding training into continuing education workshops. Improved breastfeeding education is a critical step in ensuring that health professionals are adequately prepared for this important role.

• 대한한방소아과학회지
• /
• 제31권3호
• /
• pp.46-63
• /
• 2017

Objectives The Korean Medicine (KM) PHR platform is a personalized healthcare service which allows individuals to keep and manage their own health records. When parents are reporting for their children from their memories, there is high possibility of recall errors. In these cases, it could be useful for doctors to collect the patient's symptoms through PHR platform. In this study, we aimed to investigate the clinical significance of the PHR by using the KM PHR platform in the pediatric clinic. Methods The PHR platform was used to collect child health information from parents and child care teachers. The collected data were analyzed in comparison with the results of screening by pediatrician. Results A total of 58 children were recruited, 44 of which health information were collected from their parents and their child care teachers. The remaining 14 children's health information were collected from their parents only. As a result the parents tended to perceive their children weaker than the child care teacher. Compared to other organs, there was a only significant difference in the heart weakness score and spleen weakness score in the comparison of the weak and healthy children. Conclusions Although the study was conducted on a small group of subjects, and used PHR platform developed specifically for adults to indirectly input child's symptoms, and analyzed their health information, there was a difference in health records between information providers. Development of PHR platform for children is needed to collect more reliable information.

• Child Health Nursing Research
• /
• 제8권1호
• /
• pp.55-66
• /
• 2002

It is an important task to give adequate nutritions to infants and to wean properly for rapid growth. This study was conducted to survey on doing the weaning activities of the first-time mother. This study was to provide basic data of health teaching and nursing intervention for the promotion of ideal weaning activities. Survey was done from January to February in 2001. Total 444 mothers who have first-time baby under twelve month old were interviewed in five community health center around contry (Seoul, Choung-ju, Asan, Cheon-an, Jeju). The results are summarized as follows; Starting time of weaning was common in four month old(40.4%). Most common daily weaning times by age showed once(under 3 months), twice(4 to 7 months), three times(8 to 12 months). The younger, the more commercial weaning products(p<0.01). Education level showed relatively positive relation with home-made weaning food(p<0.05). Items showed low scores in 4 to 7 months group were starting new food item, waiting at least one week interval to new food, feeding by milk bottle, allowing grasping spoon, consulting pediatrician for problems after weaning, starting from morning, regular schedule. Items showed low scores in 8 to 12 months group were feeding whole egg rather than yolk only up to 12 months, keeping weaning interval were increased, feeding by milk bottle, trying various cooking method, not giving commerical cooking and instant food and unproper raw milk feeding. In weaning activities by general factors, mother age, health status, marriage duration, monthly income were statistically significant (p<0.05). To improve good weening activities, targetting on low maternal age and poor health status, and low income group, developing teaching materials and training program for items showed low scores and proper weaning time, type of easy home-made food and easy cooking method should be provided. Further study on effect of nursing intervention to improve weaning activities are needed.

• 한국임상약학회지
• /
• 제20권3호
• /
• pp.205-212
• /
• 2010

The prescription sheets for outpatients from July 2008 to June 2009 from 7 community pharmacies in Ulsan City were surveyed for the anti-inflammatory drug (AID) prescription pattern. The AID prescription rate of pediatricians and ENT physicians were 30.0% and 34.8%, respectively. The oral steroidal anti-inflammatory drugs (SAIDs) were prescribed as much as 3.9% by pediatricians and 10.3% by ENT physicians. The chiefly prescribed oral SAID was prednisolone in pediatric clinics and methylprednisolone in ENT clinics. Meanwhile the prescription rate of oral non-steroidal anti-inflammatory drug (NSAID) was 22.5% by pediatricians and 21.4% in ENT physicians. The most favorable NSAIDs were propionate derivatives in both clinics. In case of externally-applied SAIDs, the prescription rate of pediatricians was 3.6% and that of ENT physicians was 2.8%. Among them, nasal spray, inhalant and gargle formulations for upper respiratory infection (URI) treatment occupied 35.8% of externally-applied SAIDs in pediatric clinics and 59.7% in ENT clinics. Further, it was observed that ENT physicians favored much stronger SAIDs in Group III of ATC classification (75.4% of externally-applied SAIDs) than pediatricians (49.2%). In the survey of AID combination rate, pediatric clinics showed much lower rate (1.4% of total AID prescriptions) than ENT clinics (7.5%). Among them, the combination rate of oral SAID and oral NSAID by ENT physicians (52.2% of total AID combinations) was much higher than pediatricians (36.6%), which might be over-prescription of AID agents. In conclusion, the AID prescription rate as well as AID combination rate, especially in SAID prescriptions, was much higher in ENT than pediatric clinics, which implies the higher confidency on AID drugs of ENT physicians even though the severity of patient's symptom could be considered.

• Asian Pacific Journal of Cancer Prevention
• /
• 제16권17호
• /
• pp.7737-7742
• /
• 2015

Background: Cancer is one of the common causes of disability and mortality in the world. The present study aimed to define the spatiotemporal distribution of gastrointestinal tract cancers using a geographic information system (GIS) over the time period of 2007-2012 in Kermanshah-Iran. Materials and Methods: The method of studying was descriptive-analytical as well as comparative with gastrointestinal tract cancer patients based in the City of Kermanshah over the time period covered. For data analysis, the GIS and SPSS 16.0 were applied. Results: According to the pathological reports within the space of 5 years, 283 cases of gastrointestinal tract cancer (157 in males, 156 in females) were reported. The performed tests in terms of spatial distribution in the environment of GIS indicated that the disease demonstrated a clustered pattern in the City of Kermanshah. More to the point, some loci of this disease have emerged in the City of Kermanshah that in the first level, 6 neighborhoods with 29-59 cases of this disease per square kilometer and in the second level, 15-29 cases. Conclusions: Gastrointestinal tract cancer demonstrated an ascending trend within the space of 5 years of research and the spatiotemporal distribution of cancer featured a concentrated and clustered pattern in the City of Kermanshah.

• 대한한방소아과학회지
• /
• 제24권1호
• /
• pp.104-116
• /
• 2010

Objectives The Medical record consist of symptoms, the cause of a disease, diagnostic studies, medical treatment, prescription, and so on. Doctors can use medical records as data for diagnosis, treatment, and education. Therefore, medical records are beneficial. Methods Translating $\ll$Xiao Er Yao Zheng Zhi Jue$\gg$ into modern Korean, and studying the medical records of Qian Yi(錢乙)'s Medical concept in $\ll$Xiao Er Yao Zheng Zhi Jue$\gg$ were examined. Results Regarding the diagnosis of the pediatric diseases, Si Zhen He Can(四該合參), consider in the eye exam closely. In addition, he closely combined the concept of Wu Zang Bian Zheng(五藏辨證), and diagnosis the pediatric diseases. Wu Zang Bian Zheng(五藏辨證). what Qian-Yi(錢乙) established method was based on Zheng Ti Guan(整體觀) in Huang Di Nei Jing(黃帝內經). Qian-Yi(錢乙) took a close look on Chuang Zhen(瘡疹) which had been an inadequate field in those days. He criticized on the habitual misuse of medications. He treated Ji Jing(急驚) and Man Jing(慢驚) separately; Ji Jing(急驚) as Liang Xie(凉瀉) and Man Jing(慢驚) as Wen Bu(溫補). He proposed Zong Gan Zhu Feng and Xin Zhu Jing(縱肝主風, 心主驚) theory and formulated Jing Feng(驚風) theory as well. He emphasized the treatment to Gong Bu Shang Zheng, Bu Bu Zhi Xie, Xiao Bu Jian Shi(攻不傷正, 補不滯邪, 消補兼施) because he had a clear demonstration to Han Re Xu Shi(寒熱虛實) of the five viscera in the field of Fang yao(方藥). Conclusions Understanding $\ll$Xiao Er Yao Zheng Zhi Jue$\gg$ and its medical records of Qian Yi(錢乙)'s medical concept had quintessential meaning to both modern pediatrician and oriental medical doctors of all medical fields in treating children.

• 대한소아치과학회지
• /
• 제32권4호
• /
• pp.589-594
• /
• 2005

혈우병은 성염색체 열성 유전양식에 따라 유전되는 출혈성 질환으로서 응고인자의 결핍이나 결손에 의해 혈액응고 시간이 지연되게 된다. 과거에는 혈우병 환자의 치과치료시 과다출혈의 위험성 때문에 치과시술을 위해서는 입원하여 내과 전문의나 혈액 전문의가 출혈을 조절하도록 하여 치료하는 것이 바람직한 것으로 여겨졌으나, 최근의 혈액응고기전에 대한 이해와 의료기술 발전에 의해 출혈을 좀더 효과적으로 조절할 수 있게 되었다. 본과에 내원한 혈우병 B, 중증과 혈우병 A, 중증에 해당하는 환아에서의 관혈적인 치과치료를 시행하여 각각의 과정 및 치료원칙에 관하여 다음과 같이 보고하는 바이다. 1 혈우병 환자의 치료시에는 반드시 환자의 내과 소아과 또는 혈액 전문의와 상의하여 긴밀한 협조하에 치과 치료를 시행하도록 한다. 2. 환자의 치료방법은 환자의 전신상태와 협조도 및, 필요한 시술의 위험성 정도에 따라 결정하도록 한다. 3. 치과치료의 전과 후에 혈액응고인자의 투여 등으로 발생하는 불편과 합병증의 기회를 최소로 하기 위해 치료를 위한 내원 횟수를 가능한 한 줄여주는 것이 바람직하며, 치료 시에는 감염을 예방하고 지혈을 도울 수 있는 치료를 시행해야 한다.