PURPOSE:Hypochondroplasia is a skeletal dysplasia characterized by poor childhood growth and an inadequate pubertal growth spurt. Final height attainment of hypochondroplasia has been reported to range between 120 and 152cm. Increased availability of growth hormone with the introduction of recombinant human growth hormone has allowed for clinical trials in a number of growth hormone sufficient children with growth problems. The purpose of this study was to assess the growth promoting effect of human growth hormone in children with hypochondroplasia. METHODS:Five patients with hypochondroplasia diagnosed by clinical and radiological findings between 1993 and 1997 at our hospital was aged 3 and 1/2 -11 and 1/2 years. Each patients continuously received human growth hormone 0.6-0.7U/Kg/week, intramuscularly or subcutaneously in 6-7 divided dose for 2 years. Standard auxologic assessment was carried out every 3 month interval in the first year after commencement of therapy and then same assessment was 6 monthly. Bone age was assessed 6 monthly using Gleurich-Pyle method. RESULTS:Mean height velocity of pretreatment and year 1 and 2 of GH treatment were 3.9+/-0.7, 6.5+/-1.8 and 5.7+/-1.5cm/year, respectively. Mean height standard deviation score for chronological age of pretreatment and year 1 and 2 of GH treatment were -2.7+/-0.3, -2.4+/-0.3 and -2.2+/-0.4, respectively. The increase in the height velocity diminishes over the subsequent year. The increment of bone age after GH treatment were same as the increments of chronological age. CONCLUSION: Short-term GH therapy increases the height velocity of children with hypochondroplasia, but the effect of GH therapy on final height remains unknown.
Child* ; Growth Hormone* ; Human Growth Hormone ; Humans

PURPOSE: To evaluate the immediate effects of 3% diquafosol ophthalmic solution on tear MUC5AC concentration and corneal wetting property in rabbit eyes. METHODS: Six New Zealand white rabbits were used in the present study. Fifteen minutes after instilling 50 microL of 3% diquafosol into the right eye of each rabbit and 50 microL of saline into the left eye, corneal wetting property, tear MUC5AC concentrations and the area of periodic acid-Schiff (PAS)-stained conjunctival goblet cells were evaluated under general anesthesia using conjunctival impression cytology. Corneal wetting property was evaluated by measuring the duration from when the image of the microscopic light beam was clear on the corneal surface immediately after blinking to when the image began to blur. RESULTS: The mean time of corneal wetting property was 86.40 (+/- 17.90) seconds in the diquafosol group and 49.00 (+/- 6.35) seconds in the control group. There was a significant difference between the two groups (p = 0.043). The mean concentration of tear MUC5AC was significantly higher in the diquafosol group (18.21 +/- 1.52 ng/mL) than the control group (12.75 +/- 1.82 ng/mL; p = 0.028). Conjunctival impression cytology showed the area of PAS-stained conjunctival goblet cells was significantly lower in the diquafosol group (23.17 +/- 0.05%) than the control group (32.49 +/- 0.08%; p = 0.028). CONCLUSIONS: Immediately after instilling 3% diquafosol, corneal wetting property improved significantly. Also tear MUC5AC concentration, which was released from conjunctival goblet cells increased compared to saline.
Anesthesia, General ; Blinking ; Goblet Cells ; Rabbits* ; Tears

Medullary respiratory centers are composed of two respiratory groups: dorsal and ventral. A dorsal respiratory group consists primarily of inspiratory neurons. A ventral respiratory group consists both of inspiratory and expiratory neurons. A direct infarction of their structure may lead to a complete loss of respiratory drive involving both automatic and voluntary components. A 78-year-old man was admitted with sudden dysarthria, dizziness, and bilateral ophthalmoplegia. On the second and third hospital day, he nearly had a complete loss of respiratory drive involving both automatic and voluntary components. He did not get the respiratory drive during CO2 retention while consciousness and motor were preserved. Brain MRI showed unilateral lesions involving the medullary reticular formation, nucleus tractus solitarius, nucleus ambiguus, and nucleus retroambiguus but sparing the corticospinal tract. Unilateral medullary infarction may lead to severe respiratory failures not limited to automatic responses, which differentiates it from Ondine's curse.
Aged ; Brain ; Cerebral Infarction ; Consciousness ; Dizziness ; Dysarthria ; Humans ; Infarction ; Magnetic Resonance Imaging ; Medulla Oblongata ; Neurons ; Ophthalmoplegia ; Pyramidal Tracts ; Respiratory Center ; Respiratory Insufficiency* ; Reticular Formation ; Solitary Nucleus

OBJECTIVE: To compare apparent diffusion coefficient (ADC) of the upper abdominal organs acquired with multiple b-value combinations and to investigate usefulness of normalization. MATERIALS AND METHODS: We retrospectively analyzed data, including 3T diffusion-weighted images, of 100 patients (56 men, 44 women; mean age, 63.9) that underwent liver magnetic resonance imaging. An ADC map was derived with the following six b-value combinations: b1 = 0, 50, 400, 800; b2 = 0, 800; b3 = 0, 50, 800; b4 = 0, 400, 800; b5 = 50, 800; and b6 = 50, 400, 800 s/mm2. ADC values of the right liver lobe, left liver lobe, spleen, pancreas, right kidney, and left kidney were measured. ADC values of the spleen were used for normalization. Intraclass correlation coefficients (ICCs), comparison of dependent ICCs, and repeated-measures analysis of variance were used for statistical analysis. RESULTS: Intraclass correlation coefficients of the original ADC revealed moderate to substantial agreement (0.5145–0.6509), while normalized ADCs revealed almost perfect agreement (0.8014–0.8569). ICC of normalized ADC for all anatomical regions revealed significantly less variability than that of the original ADC (p < 0.05). Coefficient of variance for normalized ADC was significantly lower than that for the original ADC (3.0.3.8% vs. 4.8.8.8%, p < 0.05). CONCLUSION: Normalization of the ADC values of the upper abdominal organs using the spleen as the reference organ significantly decreased variability in ADC measurement acquired with multiple b-value combinations.
Diffusion ; Female ; Humans ; Kidney ; Liver ; Magnetic Resonance Imaging ; Male ; Pancreas ; Retrospective Studies ; Spleen*

Ataxia-telangiectasia mutated (ATM), a master kinase of the DNA damage response (DDR), phosphorylates a multitude of substrates to activate signaling pathways after DNA double-strand breaks (DSBs). ATM inhibitors have been evaluated as anticancer drugs to potentiate the cytotoxicity of DNA damage-based cancer therapy. ATM is also involved in autophagy, a conserved cellular process that maintains homeostasis by degrading unnecessary proteins and dysfunctional organelles. In this study, we report that ATM inhibitors (KU-55933 and KU-60019) provoked accumulation of autophagosomes and p62 and restrained autolysosome formation. Under autophagy-inducing conditions, the ATM inhibitors caused excessive autophagosome accumulation and cell death. This new function of ATM in autophagy was also observed in numerous cell lines. Repression of ATM expression using an siRNA inhibited autophagic flux at the autolysosome formation step and induced cell death under autophagy-inducing conditions.Taken together, our results suggest that ATM is involved in autolysosome formation and that the use of ATM inhibitors in cancer therapy may be expanded.

OBJECTIVES: The purpose of this study was to evaluate the effectiveness of a seniors' oral health promotion program for establishing a cooperation model between public and private sectors. METHODS: This study was carried out targeting the seniors frequenting a community center in the Sosa-gu district of Bucheon-si in the Gyeonggi-do province of South Korea. Data were obtained from self-administered questionnaires or personal interviews with elders after they participated in the oral health promotion program. Their perceived satisfaction with their oral health before and after the oral health program was compared. The data were analyzed by t-tests, ANOVA, paired t-tests, and logistic regression using SPSS to assess the effects of explanatory factors on the seniors' satisfaction of the oral health promotion program. RESULTS: In total, 22.7% of participants had a positive idea about the oral health promotion program before their participation. The mean score of change in and difference of satisfaction with oral health tended to increase in each of the following groups: women, aged 80-84 years, number of permanent teeth, full denture wearers, and those who did not require dentures. Satisfaction notably increased 0.4 points in the group of full denture wearers (P<0.05). Moreover, after 1 month, the satisfaction level of the subjects who participated in the oral health promotion program increased 5.2 times (95% CI, 1.04-25.49) compared to dissatisfaction levels. Ultimately, satisfaction with oral health shows a major impact on the satisfaction with an oral health promotion program. CONCLUSIONS: The subjects who received the oral health promotion program responded very positively, but this program needs overall consideration about work processes and further reliable evaluations.
Dental Restoration Wear ; Dentures ; Female ; Gyeonggi-do ; Humans ; Korea ; Logistic Models ; Oral Health* ; Private Sector* ; Surveys and Questionnaires ; Tooth

About 90% of primary gastrointestinal lymphomas originate from the B-cell and less than 10% from the T-cell. In respect of anatomical location, the stomach is the most common site of gastrointestinal lymphomas followed by the ileum, colon, and rectum. However, esophagus and duodenal lymphomas are infrequently involved. Primary T-cell lymphoma of the duodenum is not common and peripheral T-cell lymphoma of the duodenum is very rare. In Korea, there has been no case report of peripheral T-cell lymphoma which simultaneously involved the stomach and duodenum. In this report, we present a case of primary peripheral T-cell lymphoma of the duodenum and stomach. A 63-year-old man was hospitalized complaining of weight loss of 15 kg and dyspepsia for 2 months. Esophagogastroduodenoscopy showed a large annular infiltrative lesion in the descending portion of the duodenum. At the posterior side of the upper body of the stomach, an ill-defined, broad, flat, and infiltrative lesion was also noted. Microscopic examination of the biopsy specimen showed that atypical bizzare lymphocytes infiltrated the mucosa of the duodenum and stomach. The lymphocyte was positive for CD3, CD5 and negative for CD20, CD23, and CD56 by immunohistochemistry. We made a diagnosis of primary peripheral T-cell lymphoma of the stomach and duodenum. We report a case of peripheral T-cell lymphoma of the stomach and duodenum with a review of the literature.
B-Lymphocytes ; Biopsy ; Colon ; Diagnosis ; Duodenum* ; Dyspepsia ; Endoscopy, Digestive System ; Esophagus ; Humans ; Ileum ; Immunohistochemistry ; Korea ; Lymphocytes ; Lymphoma ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral* ; Middle Aged ; Mucous Membrane ; Rectum ; Stomach* ; T-Lymphocytes ; Weight Loss

Short Bowel Syndrome (SBS) is a condition that causes malabsorption and nutrient deficiency because a large section of the small intestine is missing or has been surgically removed. SBS may develop congenitally or from gastroenterectomy, which often change the motility, digestive, and/or absorptive functions of the small bowel. The surgical procedure for SBS and the condition itself have high mortality rates and often lead to a range of complications associated with long-term parenteral nutrition (PN). Therefore, careful management and appropriate nutrition intervention are needed to prevent complications and to help maintain the physiologic integrity of the remaining intestinal functions. Initial postoperative care should provide adequate hydration, electrolyte support and total parenteral nutrition (TPN) to prevent fatal dehydration. Simultaneously, enteral nutrition should be gradually introduced, with the final goal of using only enteral nutrition support and/or oral intake and eliminating TPN from the diet. A patient should be considered for discharge when macro and micronutrients can be adequately supplied through enteral nutrition support or oral diet. Currently, there is more research on pediatric patients with SBS than on adult patient population. A 35-year-old man with no notable medical history was hospitalized and underwent a surgery for acute appendicitis at a local hospital. He was re-operated on the 8th day after the initial surgery due to complications and was under observation when he suddenly complained of severe abdominal pain and high fever. He was immediately transferred to a tertiary hospital where the medical team discovered free air in the abdomen. He was subsequently diagnosed with panperitonitis and underwent an emergency reoperation to explore the abdomen. Although the patient was expected to be at a high risk of malnutrition due to short bowel syndrome resulting from multiple surgeries, through intensive care under close cooperation between the medical and nutrition support team, his nutritional status improved significantly through continuous central and peripheral parenteral nutrition, enteral nutrition, and oral intake. The purpose of this paper is to report the process of the patient's recovery.
Abdomen ; Abdominal Pain ; Adult ; Appendicitis ; Dehydration ; Diet ; Emergencies ; Enteral Nutrition ; Fever ; Humans ; Critical Care ; Intestine, Small ; Malnutrition ; Micronutrients ; Mortality ; Nutritional Status ; Nutritional Support* ; Parenteral Nutrition ; Parenteral Nutrition, Total ; Postoperative Care ; Reoperation ; Short Bowel Syndrome* ; Tertiary Care Centers

The elastin metabolism in systemic sclerosis (SSc) has been known to be abnormal. The authors investigated relationship between the clinical manifestations of systemic sclerosis (SSc) and serum levels of soluble elastin-derived peptide (S-EDP) and anti-elastin antibodies. Serum samples were obtained from 79 patients with SSc and 79 age- and sex-matched healthy controls. Concentrations of serum S-EDP and anti-elastin antibodies were measured by ELISA. The serum concentrations of S-EDP in SSc patients were significantly higher than in healthy controls (median, 144.44 ng/mL vs 79.59 ng/mL, P < 0.001). Serum EDP concentrations were found to be correlated with disease duration in SSc (P = 0.002) and particularly in diffuse cutaneous SSc (P = 0.005). Levels of anti-elastin antibodies were found to be more elevated in SSc patients than in healthy controls (median, 0.222 U vs 0.191 U, P = 0.049), more increased in diffuse cutaneous SSc than limited cutaneous SSc (median, 0.368 U vs 0.204 U, P = 0.031). In addition, levels of anti-elastin antibodies were also found to be negatively associated with presence of anti-centromere antibody (P = 0.023). The S-EDP levels were not found to be correlated with levels of anti-elastin antibodies. The increased S-EDP and anti-elastin antibody levels and association with clinical and laboratory characteristics may reflect the abnormal metabolism in SSc.
Adult ; Antibodies, Anti-Idiotypic/*blood/immunology ; Centromere/immunology ; Elastin/*blood/immunology ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Male ; Middle Aged ; Peptides/*blood/immunology ; Scleroderma, Systemic/*metabolism/pathology

BACKGROUND AND OBJECTIVES: Laser cordectomy is considered one of the best techniques for the treatment of early stage glottic cancer. But voice outcome and quality of life (QOL) after laser cordectomy is controversial. The aim of this study is to evaluate the voice outcome after laser cordectomy in glottic cancer and its association to QOL. SUBJECTS AND METHOD: 43 patients who underwent laser cordectomy from January 2002 to December 2008 were enrolled in this study. We evaluated the objective and subjective voice outcomes using acoustic analysis, aerodynamic study and voice handicap index (VHI). We also assessed QOL using EORTC QLQ-C30/H&N35 questionnaire. We compared the voice outcome according to the type of laser cordectomy and analyzed correlation between voice outcome and QOL. RESULTS: Harmonic to noise ratio, maximum phonation time and VHI of patients treated with Types I, II cordectomy was significantly improved after operation. Functional VHI after Types I, II cordectomy was significantly lower than Types III-VI cordectomy. The postoperative QOL score of glottic cancer patients were not statistically different between Type I, II and Type III-VI cordectomy. QOL score had a positive correlation with postoperative VHI. CONCLUSION: Objective and subjective voice outcomes after laser cordectomy showed favorable results, especially in patients who were treated with Types I, II laser cordectomy. QOL scores were correlated with VHI scores, but not with acoustic parameters.
Acoustics ; Humans ; Laryngeal Neoplasms ; Microsurgery ; Noise ; Phonation ; Quality of Life* ; Surveys and Questionnaires ; Voice*