Objective: : Endothelial colony-forming cells (ECFCs) have been reported to play an important role in the pathogenesis of moyamoya disease (MMD). We have previously observed stagnant growth in MMD ECFCs with functional impairment of tubule formation. We aimed to verify the key regulators and related signaling pathways involved in the functional defects of MMD ECFCs. Methods: : ECFCs were cultured from peripheral blood mononuclear cells of healthy volunteers (normal) and MMD patients. Low-density lipoproteins uptake, flow cytometry, high content screening, senescence-associated β-galactosidase, immunofluorescence, cell cycle, tubule formation, microarray, real-time quantitative polymerase chain reaction, small interfering RNA transfection, and western blot analyses were performed. Results: : The acquisition of cells that can be cultured for a long time with the characteristics of late ECFCs was significantly lower in the MMD patients than the normal. Importantly, the MMD ECFCs showed decreased cellular proliferation with G1 cell cycle arrest and cellular senescence compared to the normal ECFCs. A pathway enrichment analysis demonstrated that the cell cycle pathway was the major enriched pathway, which is consistent with the results of the functional analysis of ECFCs. Among the genes associated with the cell cycle, cyclin-dependent kinase inhibitor 2A (CDKN2A) showed the highest expression in MMD ECFCs. Knockdown of CDKN2A in MMD ECFCs enhanced proliferation by reducing G1 cell cycle arrest and inhibiting senescence through the regulation of CDK4 and phospho retinoblastoma protein. Conclusion : Our study suggests that CDKN2A plays an important role in the growth retardation of MMD ECFCs by inducing cell cycle arrest and senescence.

Background: The aim of this study was to examine the lifestyle characteristics of hypertensive patients and to investigate the association between the duration of hypertension and lifestyle characteristics. Methods: A total 16,508 adults (including 5,933 hypertensive patients) aged 20 years and older who participated in the 7th Korea National Health and Nutrition Examination Survey 2016–2018 were analyzed. Multiple logistic regression analysis was performed to examine the association between the duration of hypertension and lifestyle factors. Results: The longer duration of hypertension was associated with lower risk of drinking in men (odds ratio [OR], 0.78; 95% confidence interval [CI], 0.66–0.93), and this observation remained when participants with hypertension for ≥ 13 years were compared to those with hypertension for < 1 year (OR, 0.57; 95% CI, 0.37–0.88). On the other hand, increased duration of hypertension increased the odds of engaging in high physical activity (PA) in women (OR, 1.72; 95% CI, 1.15–2.56), particularly in men with hypertension for 6–12 years (OR, 1.66; 95% CI, 1.03–2.69) and women with hypertension for ≥ 13 years (OR, 3.64; 95% CI, 1.38–9.62). Conclusion In conclusion, adoption and maintenance of healthy lifestyle habits, particularly drinking cessation in men, and PA in both men and women was observed, and differences were observed depending on the duration of hypertension.

BACKGROUND AND PURPOSE: This study investigated the seizure recurrence rate and potential predictors of seizure recurrence following antiepileptic drug (AED) withdrawal after resective epilepsy surgery in children with focal cortical dysplasia (FCD). METHODS: We retrospectively analyzed the records of 70 children and adolescents with FCD types I, II, and IIIa who underwent resective epilepsy surgery between 2004 and 2015 and were followed for at least 2 years after surgery. RESULTS: We attempted AED withdrawal in 40 patients. The median time of starting the AED reduction was 10.8 months after surgery. Of these 40 patients, 14 patients (35%) experienced seizure recurrence during AED reduction or after AED withdrawal. Half of the 14 patients who experienced recurrence regained seizure freedom after AED reintroduction and optimization. Compared with their preoperative status, the AED dose or number was decreased in 57.1% of patients, and remained unchanged in 14.3% after surgery. A multivariate analysis found that incomplete resection (p=0.004) and epileptic discharges on the postoperative EEG (p=0.025) were important predictors of seizure recurrence after AED withdrawal. Over the mean follow-up duration of 4.5 years after surgery, 34 patients (48.6% of the entire cohort) were seizure-free with and without AEDs. CONCLUSIONS: Children with incomplete resection and epileptic discharges on postoperative EEG are at a high risk of seizure recurrence after drug withdrawal. Complete resection of FCD may lead to a favorable surgical outcome and successful AED withdrawal after surgery.
Adolescent ; Anticonvulsants ; Child* ; Electroencephalography ; Epilepsy ; Follow-Up Studies ; Freedom ; Humans ; Malformations of Cortical Development* ; Multivariate Analysis ; Recurrence* ; Retrospective Studies ; Seizures*

Gastric hemangioma in the neonatal period is a very rare cause of upper gastrointestinal bleeding. We present a case of hemangioma limited to the gastric cavity in a 10-day-old infant. A huge, erythematous mass with bleeding was observed on the lesser curvature side of the upper part of the stomach. Surgical resection was ruled out because the location of the lesion was too close to the gastroesophageal junction. Medical treatment with intravenous H₂ blockers, octreotide, packed red blood cell infusions, local epinephrine injection at the lesion site, application of hemoclip, and gel-form embolization of the left gastric artery did not significantly alter the transfusion requirement. Hemostasis was achieved with endoscopic argon plasma coagulation (APC). After two sessions of APC, complete removal of the lesion was achieved. APC was a simple, safe and effective tool for hemostasis and the ablation of gastric hemangioma without significant complications.
Argon Plasma Coagulation* ; Argon* ; Arteries ; Epinephrine ; Erythrocytes ; Esophagogastric Junction ; Hemangioma* ; Hemorrhage ; Hemostasis ; Humans ; Infant ; Infant, Newborn* ; Octreotide ; Stomach

PURPOSE: The aim of this study was to investigate the clinical features and extraintestinal manifestations (EIMs) of Crohn disease (CD) in Korean pediatric patients. METHODS: The medical records of 73 children diagnosed with CD were retrospectively reviewed. Data regarding baseline demographic and clinical characteristics, including CD phenotype at diagnosis based on the Montreal classification, and clinical features and course of EIMs were investigated. RESULTS: Fifty-two (71.2%) of the patients were males. The mean age of the patients was 12.5 years. The mean follow-up period was 3.4 years. The disease location was ileal in 3 (4.1%) of the patients, colonic in 13 (17.8%), ileocolonic in 56 (76.7%). The clinical behavior was inflammatory in 62 (84.9%) of the patients, stricturing in 8 (11.0%), and penetrating in 3 (4.1%). Perianal abscesses or fistulas were found in 37 (50.7%) of the patients. EIMs observed during the study period were anal skin tag in 25 patients (34.2%), hypertransaminasemia in 20 (27.4%), peripheral arthritis in 2 (2.7%), erythema nodosum in 2 (2.7%), vulvitis in 1 (1.4%), uveitis in 1 (1.4%), and pulmonary thromboembolism in 1 (1.4%). CONCLUSION: Perianal diseases and manifestations were present in more than half of Korean pediatric CD patients at diagnosis. Inspection of the anus should be mandatory in Korean children with suspicious CD, as perianal fistulas, abscesses, and anal skin tags may be the first clue to the diagnosis of CD.
Abscess ; Anal Canal ; Arthritis ; Child* ; Classification ; Colon ; Crohn Disease* ; Diagnosis ; Erythema Nodosum ; Fistula ; Follow-Up Studies ; Humans ; Male ; Medical Records ; Phenotype ; Pulmonary Embolism ; Retrospective Studies ; Skin ; Uveitis ; Vulvitis

PURPOSE: To assess the feasibility and effectiveness of transperineal ultrasonography (TPUS) for the evaluation of perianal Crohn disease (PCD) in pediatric patients. METHODS: Between September 2010 and August 2013, 64 TPUS examinations were performed in 43 patients (34 males and 9 females; mean age+/-standard deviation, 13.3+/-2.4 years; age range, 6 to 17 years) to evaluate PCD. The pain severity, location, and activity of perianal fistula, the presence of an abscess, and anal canal hyperemia were retrospectively evaluated. Spearman rank correlation analysis was performed to assess the relationship between the severity of the pain and the fistula activity, the presence of an abscess, and anal canal hyperemia. RESULTS: All examinations were successfully performed. Thirty-nine examinations (60.9%) were performed without any pain experienced by the patient, 19 examinations (29.7%) with mild pain, five examinations (7.8%) with moderate pain, and one examination (1.6%) with severe pain. The pain severity was correlated with the fistula activity (P<0.01). An anterior fistula location was more common than a posterior location. Active fistulas and abscesses were identified during 30 examinations (46.9%) and 12 examinations (18.8%), respectively. Anal canal hyperemia was identified in 31 examinations (48.4%). CONCLUSION: TPUS with a color Doppler study is useful for visualizing a perianal fistula or abscess and for assessing its inflammatory activity in pediatric Crohn patients.
Abscess* ; Anal Canal ; Crohn Disease* ; Female ; Fistula* ; Humans ; Hyperemia ; Male ; Pediatrics ; Perineum ; Retrospective Studies ; Ultrasonography*

PURPOSE: To compare the abilities of radionuclide salivagrams (RS) and videofluoroscopic swallow studies (VFSS) to diagnose aspiration in children with aspiration pneumonia. METHODS: The records of children who were referred to the Asan Medical Center between April, 2006 and April, 2012 and who underwent both VFSS and RS to evaluate their recurrent aspiration pneumonia were reviewed (n=67). The aspiration positivity rates of the two tests were determined. The agreement between the tests was assessed by using the kappa statistic. RESULTS: VFSS was more frequently positive (n=26, 39%) than RS (n=23, 34%) (p=0.68). In the 11 children who repeat two test, Repeated examination increased positive rate in each tests (n=11), repeated RS (54%, p=1) is more frequent positive than repeated VFSS (46%, p=0.37). If a cumulative positive test had been defined as at least one positive result, the positive rate of two test was 56% (p<0.05). There was a fair agreement between RS and VFSS (kappa=0.26). CONCLUSION: The RS and VFSS positivity rates in children with aspiration pneumonia were similar but there was fair agreement between the two tests. This result suggests that these investigations to demonstrate aspiration are not interchangeable but complementary.
Child ; Humans ; Pneumonia ; Pneumonia, Aspiration

PURPOSE: This study aimed to evaluate the clinical features of juvenile polyp and the usefulness of polypectomy with entire colonoscopy in children. METHODS: We retrospectively reviewed the medical records of 83 children who were diagnosed with having juvenile polyps. RESULTS: The mean age of the patients was 6.5+/-3.7 (range 1.3-14.5 years) years. The male to female ratio was 2.1 : 1. Eighty one patients (97.6%) had hematochezia, of which the observed characteristics included red stool (74.1%), blood on wipe (13.6%). The time interval between the 1st episode of hematochezia and colonoscopy was 8.9+/-20.4 (ranged 0.1-48.0) months. The most proximal regions of colonoscopic approach were terminal ileum (96.4%). Sixty three patients (75.9%) had a solitary polyp and 20 patients (24.1%) had multiple polyps. The sites of the polyps were rectum (61.4%), sigmoid colon (23.5%). Eighteen polyps (15.1%) were found more proximal locations than rectosigmoid. The polyp size ranged from 0.3 to 5 cm. After the polypectomy, hematochezia recurred in 9 patients. Endoscopic hemostasis was performed in 2 patients due to severe bleeding. All procedures were carried out without using general anesthesia. CONCLUSION: Juvenile polyp occurred in a wide range locations and had variable sizes and numbers, suggesting that colonoscopy on the entire colon is necessary. Colonoscopic polypectomy is a simple and useful therapeutic method in children with juvenile polyp.
Child ; Colon ; Colon, Sigmoid ; Colonoscopy ; Female ; Gastrointestinal Hemorrhage ; Hemorrhage ; Hemostasis, Endoscopic ; Humans ; Ileum ; Male ; Medical Records ; Polyps ; Rectum ; Retrospective Studies

Epidermal inclusion cysts are formed by inclusion of keratinizing squamous epithelium within the dermis, resulting in a cyst filled with lamellated keratin. These benign cysts are usually very small and intradermal subcutaneous lesions. They can occur anywhere in the body although they are more common on the face, trunk, neck, extremities and scalp. Only a few cases of epidermal cysts of the breast have been reported in the literature. An epidermal inclusion cyst of the breast can result in several problems, even if the size is unusual. We encountered a case of a giant sized epidermal inclusion cyst of the breast initially mimicking a large fibroadenoma or phyllodes tumor.
Breast ; Breast Neoplasms ; Dermis ; Epidermal Cyst ; Epithelium ; Extremities ; Fibroadenoma ; Keratins ; Neck ; Phyllodes Tumor ; Scalp

Little is known about hair mineral status in fibromyalgia patients. This study evaluated the characteristics of hair minerals in female patients with fibromyalgia compared with a healthy reference group. Forty-four female patients diagnosed with fibromyalgia according to the American College of Rheumatology criteria were enrolled as the case group. Age- and body mass index-matched data were obtained from 122 control subjects enrolled during visit for a regular health check-up. Hair minerals were analyzed and compared between the two groups. The mean age was 43.7 yr. General characteristics were not different between the two groups. Fibromyalgia patients showed a significantly lower level of calcium (775 microg/g vs 1,093 microg/g), magnesium (52 microg/g vs 72 microg/g), iron (5.9 microg/g vs 7.1 microg/g), copper (28.3 microg/g vs 40.2 microg/g) and manganese (140 ng/g vs 190 ng/g). Calcium, magnesium, iron, and manganese were loaded in the same factor using factor analysis; the mean of this factor was significantly lower in fibromyalgia group in multivariate analysis with adjustment for potential confounders. In conclusion, the concentrations of calcium, magnesium, iron, and manganese in the hair of female patients with fibromyalgia are lower than of controls, even after adjustment of potential confounders.
Adult ; Body Height ; Body Mass Index ; Calcium/analysis ; Female ; Fibromyalgia/*metabolism ; Hair/*chemistry ; Humans ; Iron/analysis ; Magnesium/analysis ; Manganese/analysis ; Metals/*analysis ; Middle Aged