PURPOSE: Floppy infants or congenital hypotonia indicates decreased muscle tone in infants secondary to abnormalities of the central or the peripheral nervous system, or both. Previous literature classified its causes as those attributable to a central vs. peripheral origin; however, recent studies have introduced a newer classification describing a combined origin. We invenstigated floppy infants by applying the new etiological classification and reviewed the most common etiologies based on the age of presentation. We additionally reviewed the clinical characteristics, diagnoses, and the developmental outcomes in these infants. METHODS: We retrospectively reviewed the electronic medical charts and recruited 116 infants diagnosed with floppy infant syndrome between January 2005 and December 2016 at Severance Children's Hospital. Among these infants, 66 with a confirmed diagnosis were reviewed for the etiological classification. Information regarding developmental outcomes was obtained via phone interviews with the infants' families. RESULTS: Based on the new etiological classification, among 69 infants with a confirmed diagnosis, in 40 (34.5%) this syndrome was of central origin, in 19 (16.4%) of peripheral origin, and in 10 (8.6%) of combined origin. Prader-Willi syndrome, myotonic dystrophy, and spinal muscular atrophy were the most common disorders observed and combined hypotonia showed the poorest developmental outcome. CONCLUSION: The study states the importance of proper evaluation of etiological diagnosis and optimal intervention for developmental prognosis. The introduction of a new etiological group of combined hypotonia especially emphasizes regular monitoring and timely rehabilitative intervention in patients for the better quality of life in them as well as their caregivers.
Caregivers ; Classification* ; Diagnosis ; Humans ; Infant* ; Muscle Hypotonia ; Muscular Atrophy, Spinal ; Myotonic Dystrophy ; Peripheral Nervous System ; Prader-Willi Syndrome ; Prognosis ; Quality of Life ; Retrospective Studies

When performing septoplasty in patients with a high deviation of the nasal septum, effective correction is difficult and postoperative complications such as a saddle nose may result if the bone or cartilage is removed inordinately. Although several surgical techniques have been introduced, some are difficult to apply easily. Furthermore, the deviation may persist despite the application of surgical techniques due to the rebound memory of the remaining cartilage. This study aimed to describe a simple and safe surgical technique for crooked nasal septa with a high deviation. This method using horizontal dorsal septal incision allows easy separation of the highly deviated portion from the upper lateral cartilage. Furthermore, it is less traumatic than other methods, and predictably preserves the keystone area.

PURPOSE: This study was conducted in order to develop morning bread using powdered citrus peels and to examine the effect of its several biological activities for 12 weeks in overweight adults (BMI ≧ 23) living in dormitories of Chonbuk National University. METHODS: Control bread was prepared using the same formula except for replacing the wheat flour with 3% citrus peel powder. Subjects were classified according to two groups: Intake group of 3% citrus peel powder fortified morning bread (n = 30) and general morning bread (n = 30). RESULTS: The results of our study showed no significant effects of citrus peel powdered morning bread on anthropometric indices and biochemical characteristics among overweight adults. Despite the slight reduction in total-cholesterol, LDL-cholesterol, GOT, and GPT (p < 0.05), no significant differences in changes of these values were observed between the two groups. CONCLUSION: The short duration of our intervention might have resulted in the lack of any significant effect. Therefore, conduct of further studies with longer duration is warranted in order that the results from this study can be utilized as a basis for the development of food products with citrus peel powder.
Adult ; Bread* ; Breakfast ; Citrus* ; Coronary Artery Disease ; Flour ; Humans* ; Jeollabuk-do ; Overweight ; Powders* ; Triticum

Many studies for verifying angiogenesis have been in progress, especially in the field of abnormal vascular proliferation to explain the pathogenesis and to develop a treatment of several diseases. In our previous experiments, endothelial cell proliferations were induced by DMH stimulation in vitro, and the 177 factors(142 up- regulated and 35 down-regulated factors) were identified. Among the up-regulated factors, 9 substances (EFEMP1, CTGF, CYR61, ITGbeta1, FHL2, SERPINE1, MYC, PTTG1 and MSH6) were selected, which were related to cell proliferation and showed high signal intensities. The RNA was isolated from HUVECs at the time of 0, 6, 12, 24 hours after the DMH treatment, and RNA of control group HUVECs was also isolated. Genetic information of selected molecules was used to make primer for each, and RT-PCR was performed to analyze both groups. In control and treatment groups, each substance presented variety of manifestation degree according to time differences. EFEMP1, CTGF, CYR61, ITGbeta1, FHL2 and MYC were related to abnormal vascular proliferation steadily and SERPINE1, PTTG1 and MSH6 were related secondarily. CTGF was related to both normal and abnormal proliferation, but it played a more significant role in abnormal proliferation from earlier stage. EFEMP1, CYR61, ITGbeta1, FHL2 and MYC were similar to CTGF, although the relation appeared lately. Further study should be performed to analyze the expressions and the interactions of growth factors, which could be utilized in the new therapeutic development.
Cell Proliferation ; Dimenhydrinate ; Dimethylhydrazines ; Endothelial Cells* ; Intercellular Signaling Peptides and Proteins ; RNA ; Umbilical Veins

Purpose: In this study, we assessed the intake of nutrients and food groups and analyzed the nutrition quotient of obese children in South Korea. The hypothesis was that dietary quality and nutrient intake are related to the body mass index (BMI) of obese children. Methods: The participants included 48 children (20 boys and 28 girls). Based on the guidelines for the age-for-body mass index provided by the Korean National Growth Charts for children and adolescents, the participants were divided into 3 groups: overweight, BMI ≥ 85th percentile; obese, BMI ≥ 95th percentile; severely obese, BMI ≥ 99th percentile. Results: The energy distribution showed that the carbohydrate ratio was significantly higher in the overweight group (p < 0.01), whereas the fat ratio was significantly higher in the obese and severely obese groups (p < 0.05). Thus, in the overweight and obese groups, the percent body fat was negatively associated with the carbohydrate ratio (p < 0.05) but was positively associated with the fat ratio (p < 0.05). The Dietary Reference Intakes (DRI) data revealed that the intake levels of protein, vitamin B 1 , vitamin B 12 , and sodium were higher in all groups. Intakes of fiber, calcium, potassium, and magnesium were insufficient in all groups, and the DRI percent for protein was significantly higher in the obese and severely obese groups than in the overweight group. No significant differences were obtained in food group patterns or Nutrition Quotient (NQ) scores among the 3 groups. According to the evaluation results by food record and NQ, a significant difference was obtained in the dietary quality of obese children. Conclusion We conclude that a higher intake of fat enhances weight gain. Based on the study results, we propose that the guidelines should consider the energy distribution of carbohydrate and fat intake to prevent and control obesity among Korean children.

PURPOSE: The aim of this study was to investigate bronchodilator responsiveness (BDR) following methacholine-induced bronchoconstriction and to determine differences in BDR according to clinical parameters in children with asthma. METHODS: The methacholine challenge test was performed in 145 children with mild to moderate asthma, and the provocative concentration causing a 20% decline in FEV1 (PC20) was determined. Immediately after the challenge test, patients were asked to inhale short-acting beta2-agonists (SABAs) to achieve BDR, which was assessed as the change in FEV1% predictedx100/post-methacholine FEV1% predicted. For each subject, the asthma medication, blood eosinophil count, serum total IgE, serum eosinophil cationic protein level, and skin prick test result were assessed. RESULTS: The FEV1 (mean+/-SD) values of the 145 patients were 90.5+/-10.9% predicted, 64.2+/-11.5% predicted, and 86.2+/-11.2% predicted before and after methacholine inhalation, and following the administration of a SABA, respectively. The BDR did not differ significantly according to asthma medication, age, or gender. However, BDR in the atopy group (37.4+/-17.7%) was significantly higher than that in the non-atopy group (30.5+/-10.7%; P=0.037). Patients with blood eosinophilia (38.6+/-18.1%) displayed increased BDR compared with patients without eosinophilia (32.0+/-13.8%; P=0.037). CONCLUSIONS: In children with mild to moderate asthma, the responsiveness to short-acting bronchodilators after methacholine-induced bronchoconstriction was not related to asthma medication, but was higher in children with atopy and/or peripheral blood eosinophilia.
Adrenergic beta-Agonists ; Asthma ; Azides ; Bronchoconstriction ; Bronchodilator Agents ; Child ; Eosinophil Cationic Protein ; Eosinophilia ; Eosinophils ; Humans ; Immunoglobulin E ; Inhalation ; Methacholine Chloride ; Serotonin ; Skin

Arterial steal syndrome is a rare but serious complication that disrupts antegrade flow distal to an arteriovenous fistula (AVF) because of excess blood flow through the AVF. A 65-year-old woman with diabetes mellitus and undergoing hemodialysis was admitted for coldness and pain in the right hand ipsilateral to an AVF. AVF stenosis had developed 6 months after an upper-arm AVF operation. These manifestations developed 2 days after a successful radiological intervention for a stenotic lesion in the AVF, which became worse until the skin on her hand ulcerated. The symptoms became aggravated, particularly during dialysis. Fistulography revealed that the AVF anastomosis site was patent but blood flow toward the forearm had decreased severely. Arterial steal syndrome developing after percutaneous angioplasty for an AVF stenosis was suspected, and the AVF was ligated, which resolved the hand pain and ulceration.
Aged ; Angioplasty* ; Arteriovenous Fistula ; Constriction, Pathologic ; Diabetes Mellitus ; Dialysis ; Female ; Forearm ; Hand ; Humans ; Necrosis* ; Renal Dialysis ; Skin* ; Ulcer

PURPOSE: The measurement of fraction of nitric oxide (FeNO) is a noticeable tool that reflects airway inflammation in asthmatic patients. We wanted to find out the relationship between pulmonary function, bronchial hyperresponsiveness (AHR), blood eosinophilic inflammatory markers and FeNO level before and after methacholine bronchoprovocation test in asthmatic patients. METHODS: Fifty-five children, who visited the Allergy Clinic of Korea University Anam Hospital from March 2011 to February 2012, due to asthmatic symptoms, such as history of episodic wheezing or dyspnea during the previous year and resolved after using bronchodilators, were enrolled. We performed the baseline pulmonary function and methacholine bronchoprovocation test in the enrolled patients. Blood eosinophil counts and blood eosinophil cationic protein (ECP) were measured. FeNO levels were measured before and after the methacholine bronchoprovocation test. RESULTS: The mean FeNO levels (36.3 ppb) fell after methacholine bronchoprovocation test (25.7 ppb). Forced expiratory volume in one second (FEV1) %pred inversely correlated both with FeNO level before (R2=0.07, P=0.029) and after (R2=0.059, P=0.01) methacholine bronchoprovocation test. The provocative concentration, causing a 20% decrease in FEV1 to methacholine (methacholine PC20) inversely correlated both with FeNO levels before (R2=0.086, P=0.001) and after (R2=0.141, P=0.001) the challenge. FeNO level measured at bronchoconstriction state significantly correlated with blood eosinophil counts (R2=0.112, P=0.028). Serum ECP levels correlated FeNO level, neither before nor after bronchoprovocation. CONCLUSION: The baseline FeNO levels were higher in asthmatic children. However, FeNO levels rather decreased after methacholine induced bronchoconstriction. Repeated spirometry maneuver was considered to have an effect on reducing FeNO levels. FeNO correlated with pulmonary function, airway AHR and blood eosinophil counts.
Asthma ; Bronchoconstriction ; Bronchodilator Agents ; Child ; Dyspnea ; Eosinophil Cationic Protein ; Eosinophils ; Forced Expiratory Volume ; Humans ; Hypersensitivity ; Inflammation ; Korea ; Methacholine Chloride ; Nitric Oxide ; Respiratory Sounds ; Spirometry

No abstract available.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Carotid Artery Diseases/*complications/diagnosis/therapy ; Cerebral Angiography/methods ; Diagnosis, Differential ; Female ; Glucocorticoids/therapeutic use ; Humans ; Hydrocortisone/therapeutic use ; Hypopituitarism/diagnosis/*etiology/therapy ; Intracranial Aneurysm/*complications/diagnosis/therapy ; Magnetic Resonance Imaging ; Plasma Substitutes/administration & dosage ; Predictive Value of Tests ; Prednisolone/therapeutic use ; Sella Turcica ; Thyroid Hormones/therapeutic use ; Tomography, X-Ray Computed ; Treatment Outcome

Idiopathic acute eosinophilic pneumonia (IAEP), characterized by acute febrile respiratory failure associated with diffuse radiographic infiltrates and pulmonary eosinophilia, is rarely reported in children. Diagnosis is based on an association of characteristic features including acute respiratory failure with fever, bilateral infiltrates on the chest X-ray, severe hypoxemia and bronchoalveolar lavage fluid >25% eosinophils or a predominant eosinophilic infiltrate in lung biopsies in the absence of any identifiable etiology. We present a 14-month-old girl who was admitted to our pediatric intensive care unit because of acute respiratory distress. She had a fever, dry cough, and progressive dyspnea for 1 day. Chest X-ray showed multifocal consolidations, increased interstitial markings, parenchymal emphysema and pneumothorax. IAEP was confirmed by marked pulmonary infiltrates of eosinophils in the lung biopsy specimen. Most known causes of acute eosinophilic pneumonia, such as exposure to causative drugs, toxins, second-hand smoking and infections were excluded. Her symptoms were resolved quickly after corticosteroid therapy.
Anoxia ; Biopsy ; Bronchoalveolar Lavage Fluid ; Child ; Cough ; Dyspnea ; Emphysema ; Eosinophils ; Fever ; Humans ; Intensive Care Units ; Lung ; Pneumothorax ; Pulmonary Eosinophilia ; Respiratory Insufficiency ; Smoke ; Smoking ; Thorax