Malignant Paraganglioma in the Common Hepatic Duct / 대한내과학회지

Paragangliomas are rare extra-adrenal neoplasms of neural crest origin. The neoplasms may develop at various sites, but most are located in the para-aortic space along the sympathetic chain. A paraganglioma in the bile duct is very rare; only four cases of such tumors in the hepatic bile duct have been reported to date. Herein, we report on the first Korean case of a malignant paraganglioma in the common hepatic duct (with hepatic metastases) in a 75-year-old male. Computed tomography of the abdomen revealed a heterogeneously enhancing lesion in the common hepatic duct with dilatation of the intrahepatic ducts. After balloon sweeping, the mass exited spontaneously through the Ampulla of Vater. The mass was about 1.5 × 1.3 × 0.5 cm in its dimensions and the surface appeared to be necrotic and edematous. Microscopically, the tumor cells were arranged in a Zellballen pattern. The tumor was diagnosed as a malignant paraganglioma.

A Case of Primary Lung Cancer Mistaken for Pulmonary Metastasis of Renal Cell Carcinoma / 대한내과학회지

Renal cell carcinoma (RCC) is rare relative to other urological cancers, but relatively common overall among males. Even when primary tumors are successfully removed by surgery, metastases are often noted within a few years. On the other hand, masses found at other sites in patients with RCC may represent different primary cancers. We present the case of a 63-year-old man with a right lung mass and a left lung nodule who underwent radical right nephrectomy for RCC. We found no local recurrence of RCC in the abdomen. Despite treatment for RCC, the right lung mass increased in size. We performed a lung needle biopsy and diagnosed primary lung cancer. Postoperatively, the remaining left lung nodule also increased in size. It was diagnosed as an RCC metastasis upon biopsy and removed by wedge resection. The patient was treated with everolimus after the second surgery.

The Rate of Nasal Colonization of Methicillin-resistant Staphylococcus aureus at Admission to Intensive Care Units in a Secondary Hospital: The Importance of Nasogastric Tube / 병원감염관리

BACKGROUND: Methicillin-resistant Staphylococcus aureus (MRSA) nasal colonization has been known as a predictor of intensive care unit (ICU)-acquired MRSA infections. We performed this study to survey the nasal colonization of MRSA among the patients admitted to an ICU and analyze risk factors associated with the colonization. METHODS: A retrospective 1:1 matched case-control study was conducted with patients admitted to the ICU from March to December 2010 at Samsung Changwon Hospital. RESULTS: A total of 602 patients among 846 patients admitted to the ICU during the study period were evaluated. The prevalence of nasal MRSA colonization was 67 (11.1%) of 602. Other factors, including underlying renal disease (odds ratio [OR]=12.37, 95% confidence interval [CI] 3.60-42.54; P<0.001), MRSA infection within the previous 3 months (OR=7.43, 95% CI 1.31-42.05; P=0.023), nursing home resident within the previous 1 month (OR=6.25, 95% CI 1.82-21.53; P=0.004), surgical procedure within the previous 1 month (OR=5.93, 95% CI 1.86-18.85; P=0.003), and current use of nasogastric tube (OR=4.98, 95% CI 1.84-13.45; P=0.002) were independently associated with nasal MRSA colonization in patients admitted to ICU. CONCLUSION: A significant number of patients admitted to the ICU in a secondary hospital were colonized with MRSA. The present study showed the possible impact of the presence of a nasogastric tube on the nasal colonization by MRSA. More effective infection control procedures must be developed for patients with nasogastric tube use.

Familial Noonan Syndrome Confirmed by PTPN11 Gene Test / 대한소아내분비학회지

Noonan syndrome is characterized by short stature, mental retardation, typical facial morphology, webbed neck and congenital heart disease. Noonan syndrome can be inherited in an autosomal dominant manner but all Korean patients with Noonan syndrome have been reported as sporadic cases thus far. In approximately 50-60% of cases, the disease is caused by mutation in the PTPN11 (protein tyrosine phosphatase, nonreceptor type 11) gene on chromosome 12, encoding SHP-2 (Src homology protein-tyrosine phosphatase-2). We have experienced a boy and his father with typical clinical features of Noonan syndrome in whom Asn58Asp mutation of the PTPN11 gene were identified. To the best of our knowledge, this is the first report of genetically confirmed familial Noonan syndrome in Korea.

Clinical and Endocrinologic Characteristics of Children Referred for Precocious Puberty / 대한소아내분비학회지

PURPOSE: Precocious puberty is defined as the onset of secondary sexual characteristics before 8 year of age in girls and 9 year in boys. The purpose of this study is to analyze the spectrum of diagnoses made in a consecutive group of children referred for signs of precocious puberty and evaluate the clinical and endocrinologic characteristics of patients with precocious puberty. METHODS: The charts of all 948 children referred for evaluation of signs of precocious puberty between January 2003 and June 2007 in several referral centers were reviewed. Clinical features including age of onset, presenting symptoms, yearly growth rate, bone age advancement, weight, height, and body mass index were analysed and endocrine investigations included basal and gonadotropin releasing hormone (GnRH)-stimulated levels of luteinizing hormone (LH) and follicle stimulating hormone (FSH) as well as sex hormones. RESULTS: Of the 948 children referred for signs of precocious puberty, 915 (96.5%) were female and 33 (3.5%) were male. The final diagnoses made were early puberty (39%), premature thelarche (31%), true precocious puberty (27%) and precocious pseudopuberty (1%). The increases in yearly growth rate and bone age advancement were significant in true precious puberty group (P<0.05). The height and weight standard deviation score were significantly increased in true precious puberty and premature thelarche group compared to the average level according to gender and age (P<0.05). Endocrinologic studies showed that the level of basal LH, basal estradiol and GnRH-stimulated peak LH, peak FSH, peak LH/basal LH, peak FSH/basal FSH, peak LH/peak FSH ratio was all significantly higher in true precicous puberty group and early puberty group when compared to premature thelarche group (P<0.05). Neurogenic true precocious puberty among true precocious puberty was more common in boys (3 out of 7, 42.8%) than in girls (27 out of 253, 10.7%). Endocrinologic studies did not show any difference between idiopathic precocious puberty and neurogenic precocious puberty. CONCLUSION: The result of this study showed the proportion of true precocious puberty among the children referred for early pubertal signs was rather high. Collectively assessing all available data including detailed history, growth records, physical findings, laboratory studies and radiological imaging is important in the evaluation of a child with concerns of early pubertal maturation. Foregoing extensive studies regarding incidence and causes of true precocious puberty should be needed.

Time Trend in Height, Weight, BMI and Waist Circumference of Korean Adolescents; From the Korean National Health and Nutrition Examination Survey (KNHNES), 1998, 2001 and 2005 / 대한소아내분비학회지

PURPOSE: The mean height and weight had been increasing rapidly in Korean adolescents. The purpose of this study was to analyze body physique (height, weight, BMI and waist circumference) of Korean adolescents, using KNHNES data. METHODS: The data were obtained from the KNHNES from each year of 1998, 2001 and 2005. Total number of 4164 adolescents (2140 boys, 2024 girls) aged 10 to 19 years were included. Height, weight, and waist circumference were measured and the mean value of body physique was analyzed by sex and age. RESULTS: For height of 10 yr-old boys, there was no difference between 1998, 2001 and 2005, but the height of 13 yr-old boys was increased more than 3 cm in 2005, compared to 1998 and 2001. For height of 10 yr-old girls, more than 2 cm was increased, but, there was no significant difference after the age of 14 year in each 1998, 2001 or 2005. Height of the age of 19 was 173.7 cm in boys and 161.3 cm in girls in 2005, showing no distinct change compared to 1998 or 2001. Weight and BMI showed significant increase in both sexes and all-aged groups for older than the age of 11 years in 2005, compared to 1998 or 2001. Weight increase was more distinct in boys compared to girls. For boys, waist circumference of 2005 were higher than 1998, but lower than 2001. For girls, waist circumference of 2005 were higher than 1998 and 2001 especially in the late teens. CONCLUSION: Rapid increase in height was shown in the early teenage whereas final height showed no significant difference during 1998-2005. The mean weight and BMI have been increased dramatically in 2005, compared to 1998 or 2001 in boys and girls. This phenomenon was shown more remarkably in boys, compared to girls. These findings emphasize importance of obesity management, especially on boys.