PURPOSE: The growth hormone receptor(GHR) is essential for the actions of growth hormone on postnatal growth and metabolism. GHR transcripts are characterized by the presence of disparate 5'untranslated exons. In contrast to L1 transcript, factors regulating the expression of the GC rich L2 transcript have remained unidentified. The purpose of this study is in order to characterize the mechanisms regulating expression of the L2 transcript in the murine GHR gene METHODS: Transient transfection experiments including deletional analysis and co-transfection assay were performed to find a region containing promoter activity in the L2 5'flanking sequence using BNCL2(mouse liver) cells, CV-1(African green monkey kidney) cells, HRP.1 trophoblasts and Drosophila Schneider(SL2) cells. Sequencing analysis was performed to find the region contained consensus binding sites for transcription factors. Standard gel shift(Electrophoretic mobility shift assay, EMSA) and supershift analysis using liver nuclear extracts was performed to establish proteins(transcription factors) bound this regulatory element. RESULTS: The 5'flanking region of the L2 untranslated region(UTR) exhibited promoter activity in BNCL2(mouse liver), CV-1(monkey kidney) cells and HRP.1 trophoblasts. Deletional analyses indicated the presence of a Sp binding site important for transcription of the L2 UTR and localized the major regulatory region within 75 bp of the 5'transcription start site. Sequencing analyses revealed the region contained consensus binding sites for the Sp family of transcription factors. EMSA and supershift EMSA revealed that in mouse liver nuclear extracts that Spl and Sp3 bound to this cis-element. Functional studies in Drosophila SL2 cells and BNCL2(mouse liver) cells established the ability of Sp3 and Sp1 to stimulate transcriptional activity via this cis-element. Functional studies in Drosophila SL2 cells demonstrated a functional interaction between Sp3 and Sp1 at this DNA-binding site. CONCLUSION: Sp family transcription factors play a role in regulation of L2 transcript gene expression in the 5'flanking region of the murine GHR gene.
Animals ; Binding Sites ; Cercopithecus aethiops ; Consensus ; Drosophila ; Electrophoretic Mobility Shift Assay ; Exons ; Gene Expression ; Growth Hormone* ; Humans ; Liver ; Metabolism ; Mice ; Receptors, Somatotropin* ; Regulatory Sequences, Nucleic Acid ; Transcription Factors* ; Transfection ; Trophoblasts

Glutathione S-transferase (28GST) with molecular mass of 28 kDa is an antioxidant enzyme abundant in Clonorchis sinensis. In adult C. sinensis, 28GST was localized in tegumental syncytium, cytons, parenchyma, and sperm tails examined by immunoelectron microscopy. C. sinensis 28GST was earlier found to neutralize bioreactive compounds and to be rich in eggs. Accordingly, it is suggested that 28GST plays important roles in phase II defense system and physiological roles in worm fecundity of C. sinensis.
Animals ; Clonorchis sinensis/*enzymology ; Glutathione Transferase/*metabolism/physiology ; Immunohistochemistry ; Microscopy, Immunoelectron ; Molecular Weight ; Support, Non-U.S. Gov't

Adenotonsillar hypertrophy is the leading cause of childhood obstructive sleep apnea. Obstructive sleep apnea syndrome in child-hood, however, can occur from various causes such as obesity or craniofacial abnormalities. Childhood obstructive sleep apnea syndrome can be accompanied by enuresis, parasomnias and behavior problems. For patients with the symptoms of snoring and apnea, obstructive sleep apnea should be suspected and diagnosed properly. In addition, the evaluation of complications and proper treatment are indispensable. When the cause of childhood obstructive sleep apnea is adenotonsillar hypertrophy, symptoms can be improved by surgical methods. If the cause is other than adenotonsillar hypertrophy, such as obesity, it should be treated with other therapeutic modalities, like nasal continuous positive airway pressure (nCPAP), weight reduction and modification of life style. This paper reports a case of nCPAP used to manage severe sleep apnea when it was not resolved after adenoidectomy and tonsillectomy. Differential diagnosis of narcolepsy in a case with excessive daytime sleepiness and reflections on accompanying enuresis and parasomnia were also described.
Adenoidectomy ; Apnea ; Child ; Continuous Positive Airway Pressure ; Craniofacial Abnormalities ; Diagnosis, Differential ; Enuresis ; Humans ; Hypertrophy ; Life Style ; Narcolepsy ; Obesity ; Parasomnias ; Sleep Apnea Syndromes ; Sleep Apnea, Obstructive* ; Snoring ; Tonsillectomy ; Weight Loss

OBJECTIVES: The vocal changes after a thyroidectomy are temporary and nonsevere, therefore, obtaining accurate analytical results on the pathological vocal characteristics following such a procedure is difficult. For a more objective acoustic analysis, this study used the cepstral analysis method to examine changes in the patients' voices during the perioperative period regarding sustained vowel phonation. METHODS: The sustained phonation of the five vowels (i.e., /a/, /e/, /i/, /o/, and /u/) by 35 patients with thyroidectomy were recorded by using a Multi-Speech program. Of the 35 patients, 10 were men and 25 were women, with an average age of 51.5 years. Voice data were collected a total of 3 times (preoperatively, 5-7 days after the operation, and 6 weeks after the operation) and were edited according to each fragment (on-set, mid, and off-set) for cepstral analysis. RESULTS: The cepstral analysis on the patients' voices revealed no significant differences between the examination periods of all vowel phonations. However, analysis of the on-set fragment of the vowel /i/ revealed pathological characteristics in which the cepstral measurements of the voice were significantly lower after the operation than before the operation, with the cepstral measurements of the voice increasing further 6 weeks following surgery. CONCLUSION: The results of the acoustic analysis on the on-set fragment of the vowel /i/ will be important data for characterizing the vocal changes during the perioperative period. This study contributes to future research on the mechanisms underlying changes in the voice of patients with a history of thyroid or neck surgery.
Acoustics ; Female ; Humans ; Male ; Methods ; Neck ; Perioperative Period ; Phonation ; Thyroid Gland ; Thyroidectomy* ; Voice*

Glycogen storage diseases(GSD) are inherited disorders affecting glycogen metabolism and type I GSD is due to the absence or deficiency of glucose-6-phosphatase(G6Pase) enzyme in the liver, kidney, and intestinal mucosa. The defect leads to inadequate hepatic conversion of G6P to glucose and thus make affected individuals susceptible to fasting hypoglycemia, and the accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form of GSD and clinically growth retardation may manifest of GSD itself rather than growth hormone deficiency(GHD), but we experienced a case of type I GSD with GHD in a 14-year-o1d male. The height was 125 cm, compatible with 50 th percentile of height of 8 years of age. He has doll-like face with fat cheek, relatively thin extremities, and metabolic acidosis, hyperuricemia, hypoglycemia, hyperlipidemia. GH stimulation test with clonidine and L-dopa revealed that the patient had decreased GH secretion. After laboratory work up including liver biopsy, he was diagnosed as type I GSD. Hypoglycemia was managed with frequent feeding with high starch diet(uncooked cornstarch). Metabolic acidosis and hyperuricemia were treated with sodium bicarbonate, allopurinol and probenecid. The patient is being followed at out-patient clinic with clinical improvement after of diet therapy and GH administration.
Acidosis ; Allopurinol ; Biopsy ; Cheek ; Clonidine ; Diet Therapy ; Extremities ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone* ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Levodopa ; Liver ; Male ; Metabolism ; Outpatients ; Probenecid ; Sodium Bicarbonate ; Starch

Growth hormone receptor gene is expressed in the myocardium and growth hormone(GH) administration has been shown to increase cardiac insulin-like growth factor-I(IGF-1) contents. The cardiac myocyte express IGF-I receptors and IGF-I promotes cardiac hypertrophy. Furthermore GH therapy has been reported to have beneficial effects in the treatment of dilated cardiomyopathy in adults. We have studied the effect of short term growth hormone treatment in two cases of a 5 year-old girl and a 15 year-old boy presenting idiopathic dilated cardiomyopathy. Transthoracic echocardiography revealed that the left ventricular chamber was markedly dilated and the global left ventricular systolic function was severely reduced in both. Medical treatments such as inotropic agents, diuretics and angiotensin converting enzyme(ACE) inhibitor were tried but we could not attain remarkable improvement. Finally, we started growth hormone treatment (0.35 U/kg/week, Eutropin, LGCI, Korea) and after treatment for 3 months, we attained a remarkable hemodynamic improvement in the 5 year-old girl with remarkable IGF-I increase after GH treatment, but not in the 15 year-old boy without IGF-I increase. The treatment was safe without side effects. However, no beneficial effects on cardiac function or structure were detected at 6 months of post-treatment.
Adolescent ; Adult ; Angiotensins ; Cardiomegaly ; Cardiomyopathy, Dilated* ; Child, Preschool ; Diuretics ; Echocardiography ; Female ; Growth Hormone* ; Hemodynamics ; Humans ; Insulin-Like Growth Factor I ; Male ; Myocardium ; Myocytes, Cardiac ; Receptor, IGF Type 1 ; Receptors, Somatotropin

The risk of cardiovascular disease is known to be increased in obstructive sleep apnea syndrome (OSAS). Its mechanism can be explained by the observation that the sympathetic tone increases due to repetitive apneas accompanied by hypoxias and arousals during sleep. Heart rate variability (HRV) representing cardiac autonomic function is mediated by respiratory sinus arrhythmia, baroreflexrelated fluctuation, and thermoregulation-related fluctuation. We evaluated the heart rate variability of OSAS patients during night to assess their relationship with the severity of the symptoms. We studied overnight polysomnographies of 59 male untreated OSAS patients with moderate to severe symptoms (mean age 45.4+/- 11.7 yr, apnea-hypopnea index [AHI]=43.2+/-23.4 events per hour, and AHI >15). Moderate (mean age 47.1+/-9.4 yr, AHI=15-30, n=22) and severe (mean age 44.5 +/-12.9 yr, AHI >30, n=37) OSAS patients were compared for the indices derived from time and frequency domain analysis of HRV, AHI, oxygen desaturation event index (ODI), arousal index (ArI), and sleep parameters. As a result, the severe OSAS group showed higher mean powers of total frequency (TF) (p=0.012), very low frequency (VLF) (p= 0.038), and low frequency (LF) (p=0.002) than the moderate OSAS group. The LF/HF ratio (p=0.005) was higher in the severe group compared to that of the moderate group. On the time domain analysis, the HRV triangular index (p=0.026) of severe OSAS group was significantly higher. AHI was correlated best with the LF/HF ratio (r(p)=0.610, p<0.001) of all the HRV indices. According to the results, the frequency domain indices tended to reveal the difference between the groups better than time domain indices. Especially the LF/HF ratio was thought to be the most useful parameter to estimate the degree of AHI in OSAS patients.
Adult ; Cardiovascular Diseases/*diagnosis/pathology ; Electrocardiography/methods ; *Heart Rate ; Humans ; Male ; Middle Aged ; Oximetry ; Oxygen/metabolism ; Plethysmography ; Polysomnography/methods ; Sleep ; Sleep Apnea, Obstructive/*diagnosis/pathology

The present study compared the actigraphic indices between both wrist actigraphies (WATGs), and the sleep estimates between each WATG and nocturnal polysomnography (NPSG) to assess their differences and consistencies. We studied 22 right-handed subjects (mean age 43.9+/-13.3 years, M:F=14:8) with untreated primary sleep disorders (primary insomnia=8, simple snorer=2, obstructive sleep apnea=12) undergone by overnight both WATGs and NPSG, simultaneously. Comparison and correlation were analyzed between right and left wrist actigraphic data. In the sleep estimates of both WATGs and NPSG, each WATG was compared and correlated with NPSG in sleep period time (SPT), total sleep time (TST), sleep latency (SL), sleep efficiency (SE) and wake time (WT). Sleep indices between both WATGs showed significant positive correlations with no correlations in SL and fragmentation index (FI). There were no differences in sleep indices between both WATGs. SPTs of both WATGs, SL of left WATG, and TST of right WATG showed positively significant correlations, and SE of right WATG did negatively significant correlation in sleep indices between each WATG and NPSG. As each WATG was compared to PSG, SPTs of both WATGs and WT of right WATG were decreased, and TST and SE of right WATG and SL of left WATG were increased. Inconsistent SL and FI between both WATGs indicate that the activities between both WATGs can differentially happen during wake or arousal. Inconsistent sleep estimates between each WATG and NPSG may indicate the limited usefulness in measuring and analyzing one-night sleep by using WATG.
Arousal ; Functional Laterality ; Polysomnography* ; Sleep Wake Disorders ; Wrist*

No abstract available.
Graft Survival* ; Humans ; Living Donors* ; Risk Factors* ; Transplants*

No abstract available.
Cause of Death* ; Kidney Transplantation* ; Kidney*