A Case of Erdheim-Chester Disease Developed during Treatment of Leukemia in a Child / 임상소아혈액종양

Erdheim-Chester disease (ECD) is a rare non-Langerhan's cell histiocytosis disorder characterized by replacement of normal tissue by lipid-laden histiocytes affecting various organs. A few pediatric cases have been reported worldwide. Here we present a child with leukemia who was diagnosed as ECD. A 2-year and 11-month old boy diagnosed with high risk acute lymphoblastic leukemia (ALL) at the age of 17 months, received allogeneic hematopoietic stem cell transplantation (HSCT) at the age of 2 years old. Six months after the transplantation, the patient was admitted to the hospital with palpable left calf nodules. Bone marrow study suggested ECD without leukemia with complete chimerism status. Excisional biopsy of the left calf nodule showed ‘aggregation of non-Langerhan's cell type epitheloid histiocytes’; clinically suggestive of ECD. The patient was started on vinblastine and corticosteroid treatment.

Clinical manifestations of Rathke’s cleft cysts and their natural progression during 2 years in children and adolescents

PURPOSE: Rathke’s cleft cyst (RCC) is an asymptomatic benign lesion. With increased interest in pediatric endocrinology, the prevalence of RCCs in children is also increasing. However, the clinical relevance and proper management of RCC is not well defined in children. Therefore, we investigated the clinical manifestations and radiologic features of RCC in children and adolescents, as well as the natural progression of RCC. METHODS: We retrospectively reviewed the medical records of 91 children and adolescents with RCC diagnosed with magnetic resonance imaging (MRI) in Severance Children’s Hospital from January 2006 to December 2015. The clinical, hormonal, and imaging findings were analyzed in patient groups classified according to age. The size of each cyst was assessed in sixty patients who underwent follow-up MRI during the 2 years. RESULTS: Female patients were predominant (64 vs. 27). The common clinical features at presentation were endocrine dysfunction (59.3%), headache (23.0%), and dizziness (4.4%). Symptoms related to endocrine disorders were more frequent in younger patients. In 7 patients managed surgically, the cysts were significantly larger and more frequently located in the suprasellar region. Of 60 nonsurgical patients with a follow-up MRI performed within 2 years after the diagnosis, the RCC size increased in about 26.7% (n=16). CONCLUSION: Although 94.4% of the patients with RCC had clinical symptoms, surgery was performed in only about 7.5% of patients. RCC is associated with pituitary insufficiency, thus, baseline and follow-up endocrine function tests are required. Additionally, regular MRI follow-up is required in long-term period to monitor change in size.

Diabetes mellitus due to agenesis of the dorsal pancreas in a patient with heterotaxy syndrome

Heterotaxy syndrome (HS) is a congenital disorder resulting from an abnormal arrangement of visceral organs across the normal left-right axis in the embryonic period. HS is usually associated with multiple anomalies, including defects of the major cardiovascular system and the extracardiovascular system such as intestinal malrotation, abnormal lung lobulation, bronchus anomalies, and pancreatic dysplasia. Although pancreatic dysplasia is occasionally accompanied with HS, the occurrence of diabetes mellitus (DM) due to pancreatic dysplasia in HS is rarely reported. We here report a case involving 13-year-old girl with DM caused by agenesis of the dorsal pancreas and HS diagnosed on the basis of the presence of a double-outlet right ventricle with bilateral pulmonary stenosis and intestinal malrotation with duodenal cyst. Timely diagnosis and treatment with insulin improved glycemic control.

Validation Study of Kim's Smoking Cessation Motivation Scale and Its Predictive Implications for Smoking Cessation

OBJECTIVE: The purpose of this study was to develop a scale to measure motivation for smoking cessation. Motivation is known to be important for success of smoking cessation. The reliability of the scale was assessed and its predictive validity for smoking cessation was evaluated. METHODS: We recruited 333 men aged 20 to 70 that visited smoking cessation clinics at seven public health centers. The demographic characteristics were recorded and the Korean version of Stages of Readiness for Change and Eagerness for Treatment Scale for Smoking (K-SO-CRATES-S) performed. A smoking cessation motivation scale was developed with 10 questions based on the theory of motivation enhancement therapy. RESULTS: The motivation scale was composed of four subscales based on the factor analysis; each subscale had an adequate degree of internal consistency. In addition, the newly developed scale had a high degree of validity based on its significant correlation with the smoking version of SOCRATES. Moreover, the precontemplation level of motivation was found to significantly predict the success of smoking cessation. And one of the subscales of the Korean Nicotine Dependence Syndrome Scale (K-NDSS), stereotypy which also significantly predicted the success of smoking cessation, significantly correlated with the preparation 1 and 2 level of motivation. CONCLUSION: The smoking cessation motivation scale with 10 questions that was developed in this study was a highly reliable and valid scale for the prediction of success for smoking cessation for those who wanted to stop smoking.

Validation Study of Kim's Smoking Cessation Motivation Scale and Its Predictive Implications for Smoking Cessation

OBJECTIVE: The purpose of this study was to develop a scale to measure motivation for smoking cessation. Motivation is known to be important for success of smoking cessation. The reliability of the scale was assessed and its predictive validity for smoking cessation was evaluated. METHODS: We recruited 333 men aged 20 to 70 that visited smoking cessation clinics at seven public health centers. The demographic characteristics were recorded and the Korean version of Stages of Readiness for Change and Eagerness for Treatment Scale for Smoking (K-SO-CRATES-S) performed. A smoking cessation motivation scale was developed with 10 questions based on the theory of motivation enhancement therapy. RESULTS: The motivation scale was composed of four subscales based on the factor analysis; each subscale had an adequate degree of internal consistency. In addition, the newly developed scale had a high degree of validity based on its significant correlation with the smoking version of SOCRATES. Moreover, the precontemplation level of motivation was found to significantly predict the success of smoking cessation. And one of the subscales of the Korean Nicotine Dependence Syndrome Scale (K-NDSS), stereotypy which also significantly predicted the success of smoking cessation, significantly correlated with the preparation 1 and 2 level of motivation. CONCLUSION: The smoking cessation motivation scale with 10 questions that was developed in this study was a highly reliable and valid scale for the prediction of success for smoking cessation for those who wanted to stop smoking.