We experienced two cases of congenital adrenal hyperplasia(CAH), complicated by true precocious puberty with early maturation of the hypothalamic-pituitary-gonadal axis in a boy and a girl. The boy was diagnosed as CAH at 8 years of age, when he showed pseudoprecocity with adrenal rest tumor. The girl was diagnosed at neonate, when she showed ambiguous genitalia and salt-losing symptom. They developed precocious puberty during steroid treatment. Clinically they showed precocity, advanced bone age, and decreased predicted adult height, even though they showed partial gonadotrophin response to luteinizing hormone releasing hormone (LHRH) stimulation test. They were treated with LHRH analogue(leuprolide depot) resulting no further progression of precocity.
Adrenal Hyperplasia, Congenital* ; Adrenal Rest Tumor ; Adult ; Axis, Cervical Vertebra ; Disorders of Sex Development ; Female ; Gonadotropin-Releasing Hormone ; Humans ; Infant, Newborn ; Male ; Puberty, Precocious*

BACKGROUND: The purpose of this article was to identify the risk factors related to development of hemodynamically significant cardiac arrythmias in patients with mechanical ventilation. METHODS AND RESULTS: Holter recording and echocardiogram were performed after 30 minutes of ventilator initiation in patients on mechanical ventilation(MV) owing to respiratory failure(RF) from various reasons. From 68 patients, hemodynamically significant cardiac arrythmias were detected in 18 patients(26.5%). Initial mean arterial pressure, maximal heart rate, and initial pH were identified as risk factors for hemodynamically significant cardiac arrythmias. Additionally, the patients with pressure-controlled ventilation as an initial ventilatory mode developed hemodynamically significant cardiac arrythmias less frequently than the patients with other modes(15.8% vs. 40%, p=0.03). In multivariate analysis, initial mean arterial pressure(<70mmHg, odds ratio[OR]:5.5;95% confidence interval[CI]:1.2 to 24.2, p=0.026), maximal heart rate(>120/min, OR:19.7;95% CI:2.0 to 190.0, p=0.01), and pressure-controlled ventilation(OR:0.13,95% CI:0.03 to 0.55, p=0.006) were associated with the development of hemodynamically significant cardiac arrhythmias. CONCLUSION: Theses findings suggest that during the early stages of mechanical ventilation with acute respiratory failure, hemodynamically significant cardiac arrhythmias are directly associated with tachycardia(>120/min), initial MAP(<70mmHg), and, inversely, the initial use of pressure-controlled ventilation.
Arrhythmias, Cardiac* ; Arterial Pressure ; Heart ; Heart Rate ; Humans ; Hydrogen-Ion Concentration ; Multivariate Analysis ; Respiration, Artificial* ; Respiratory Insufficiency* ; Risk Factors* ; Ventilation ; Ventilators, Mechanical

Urinary growth hormone (GH) excretion was quantitated in 12-hr overnight urine collections obtained from 13 children with complete growth hormone deficiency (CGHD). 6 children with partial growth hormone deficiency (PGHD), 5 children with short stature and normal GH provocation tests (NSC), and 5 normal control children (NC) to investigate whether the measurement of urinary GH can clearly separate the PGHD and CGHD groups from the NSC and NC groups. In addition, the urinary excretion of GH was measured in CGHD after sc injections of 0.1 IU/kg GH three times a week and daily in an attempt to determine the optimal replacement dose. The results were as follows. 1) The 12-hr urinary GH excretion cleary separated the CGHD (1.2+/-1.1 ng/12 hr; range, 0.3-3.1, n=13) and PGHD (3.7+/-2.4 ng/12 hr; range, 0.9-6.8 n=6) groups from the NSC (14.0+/-3.6 ng/12 hr; range, 8.5-18.2 n=5) and NC (12.7+/-5.8 ng/12 hr; range, 6.9-20.8, n=5) groups without any overlap. 2) There were significant difference in 12-hr urinary GH excretion between CGHD and PGHD, but the higher values in CGHD groups overlapped some of the lower values in PGHD group. 3) There were no differencies in 12-hr urinary GH excretion between NC and NSC. 4) A 12-hr urinary GH values less than 6.9 ng/12 hr suggested GH deficiency. 5) The CGHD patients who received o.1 IU/kg GH. sc. daily GH levels within the range of the mean +/-SD in NC. but those who received theree times a week had daily variable urinary GH levels. These results suggest that the measurement of 12-hr nocturnal urinary GH excretion monitering of GH therapy response.
Child ; Growth Hormone* ; Humans ; Urine Specimen Collection

PURPOSE: There has been controversy of the lobectomy for well differentiated thyroid cancer (WDTC). Current guidelines recommend total thyroidectomy for the cancer over 1cm, despite previous report suggesting that the lobectomy provides similar excellent outcomes. The purpose of our study is to report our experience of WDTC treated by thyroid lobectomy. METHODS: We retrospectively analyzed 284 patients with WDTC treated by thyroid lobectomy at department of Surgery in Chosun University Hospital from January 2002 to December 2010. Overall survival (OS) and disease-free survival (DFS) were determined by the Kaplan-Meier method. Factors predictive of recurrence by univariate and multivariate analysis were determined using the χ2 test and Cox proportional hazard model respectively. RESULTS: With a mean follow-up of 60.4 months, OS and RFS for all patients were 97.9% and 96.5% respectively. No patient died due to WDTC. Univariate analysis showed statistically significant differences in recurrence by tumor size (P=0.013) and presence of invasion (P=0.039). However, Multivariate analysis showed no significant difference in local recurrence. CONCLUSION: Patients with WDTC confined to one lobe can be safely treated by lobectomy.
Disease-Free Survival ; Follow-Up Studies ; Humans ; Methods ; Multivariate Analysis ; Proportional Hazards Models ; Recurrence ; Retrospective Studies ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy

BACKGROUND: Serious pulmonary complications after lung surgery increase morbidity and mortality in perioperative period. Ketamine hydrochloride produces strong analgesic effect in spite of the psychomimetic effects. Intravenous anesthesia with ketamine was performed in lung surgery of patients with decreased pulmonary function and compared with inhalation anesthesia with enflurane. METHODS: Sixty patients, scheduled for elective lung surgery, were randomly assigned to two groups. Patients received either enflurane (Group 1, n=30) or ketamine (Group 2, n=30) as main anesthetic drug. Blood pressure and heart rate were compared in preinduction, postinduction, postintubation, postincision, intraoperative period (30 minutes, 60 minutes) and recovery room between groups, and in each group. Arterial blood gas analysis was compared in preoperative period, intraoperative period and recovery room between groups. Postoperative psychological complications evaluated in group 2. RESULTS: Blood pressure and heart rate were significantly different in postinduction, postintubation and recovery room between groups. PaO2 in group 2 was higher than in group 1 during intraoperative period and recovery room. Postoperative psychological complications occured in 4 patients (13%) in group 2. CONCLUSIONS: Ketamine affords advantage over enflurane anesthesia in terms of PaO2 during intraoperative period and recovery room in lung surgery of patients with decreased pulmonary function.
Anesthesia ; Anesthesia, Inhalation* ; Anesthesia, Intravenous* ; Blood Gas Analysis ; Blood Pressure ; Enflurane* ; Heart Rate ; Humans ; Inhalation* ; Intraoperative Period ; Ketamine* ; Lung* ; Mortality ; Perioperative Period ; Preoperative Period ; Recovery Room

BACKGROUND: In animal models of cerebral ischemic-reperfusion has been shown to have a beneficial effect. The object of this study is to compare the effect of pathologic findings between normotheimic and moderate hypothermic group. METHODS: We investigated the effect of moderate hypothermia induced 1 hour after transient(10 min) both carotid artery occlusion on the extent of ischemic-reperfusion cell damage in Mongolian Gerbil model. The terminal deoxyribonucleotidyl transferase (TdT) -mediated biotin-16-dUTP nick-end labelling(TUNEL staning) are used to detect apoptosis. RESULTS: 1. We suggest that Core body temperature is down to moderate hypothermia(30-32degrees C) beyond 10 minite by selective bain cooling method in Mongolian Gerbil model. 2. By light microscopy, ischemic-reperfusion damage were detected in the hippocampal CA1 pyramidal layer on the 3 day after transient ischemic insult, which showed chrosomal condensation and cytoplasmic eosinophilia. Ischemic-reperfusion cells were increased in the CA1 region on the 5 day. Apoptotic cells of the CA1 neurons seen by TUNEL staining than ischemic neurons seen by Hematoxylin-eosin staining were investigate 3 and 5 days after ischemic-reperfusion insult. CONCLUSION: We suggest that is not neuroprotective effects of Intraischemic(1 hour) moderate hypothermia in Gerbil brain global ischemic-reperfusion model.
Apoptosis ; Body Temperature ; Brain* ; Carotid Arteries ; Cytoplasm ; DNA Nucleotidylexotransferase ; Eosinophilia ; Gerbillinae* ; Hypothermia* ; In Situ Nick-End Labeling ; Microscopy ; Models, Animal ; Neurons ; Neuroprotective Agents*

PURPOSE:Autonomic neuropathy in adult diabetics is known to be common and poor in prognosis. Cardiac autonomic neuropathy increases sudden cardiac death and is present before apparant cardiac symptoms. Subclinical diabetic peripheral neuropathy is common with type 1 diabetes. In children, few studies for subclinical peripheral neuropathy and cardiac autonomic neuropathy were reported but the results were inconsistent. The authors investigated subclinical cardiac and peripheral neuropaties and evaluated correlation between nerve conduction velocity(NCV) and cardiac autonomic function(CAF) test in type 1 diabetic children and adolescents METHODS:Over 5 years from 1997 to 2002, 27 IDDM without clinical neuropathy were registered. Total 57 CAF and NCV were tested every 2 or 3 years. Duration of diabetes, average HbA1c during 1 year prior to the test, and urinary microalbumin excretion for 24 hours were assessed. Diabetic autonomic nervous function was evaluated by cardiovascular reflex test:falling systolic blood pressure in response to standing, heart rate changes in response to standing, beat-to-beat rate variation during deep breathing, RR interval change during and after Valsalva maneuver. Subclinical peripheral neuropathy was evaluated by NCV in both upper and lower extremities. Correlation of parameters according to NCV abnormality and microalbuminuria were analyzed. RESULTS:The age, duration of diabetes and microalbuminuria were not statistically different between children with normal and abnormal NCV. The level of HbA1c was associated with NCV abnormality. In a multivariate logistic model for NCV abnormality, level of HbA1c showed statistical significance after controlling the effects of age, duration of diabetes, and urinary microalbumin level, and the odd ratio was 1.532. The degree of CAF abnormality was marginally significant in the logistic model. Children with microalbuminuria showed older age and longer duration of diabetes than those without microalbuminuria. However, mean HbA1c level was not significantly different between the two groups. Degree of CAF abnormality was not significantly associated with microalbuminuria, either. In a general linear model including urinary albumin level as the dependent variable, none of independent variables was statistically significant. CONCLUSION: Therapy against neuropathy should be considered in patients with high HbA1c and abnormal CAF test even without clinically apparent neuropathy.
Adolescent ; Adult ; Blood Pressure ; Child* ; Death, Sudden, Cardiac ; Diabetes Mellitus, Type 1 ; Diabetic Neuropathies ; Heart Rate ; Humans ; Linear Models ; Logistic Models ; Lower Extremity ; Neural Conduction ; Peripheral Nervous System Diseases ; Prognosis ; Reflex ; Respiration ; Valsalva Maneuver

Recent studies suggest the flow cytometric DNA ploidy analysis may be useful in defining the biologic behavior and prognosis in prostatic adenocarcinoma. Flow cytometric nuclear DNA ploidy analysis was used to study the relationship between DNA ploidy, clinical stage and histopathological grade in thirty two patients with prostatic adenocarcinomas diagnosed from 1987 to 1990. The incidence of aneuploidy in the total population was 18 of 32 (56.3%). The frequency of aneuploidy increased with advancing stage and 63.2% of carcinomas with distant metastases were aneuploidy. Aneuploidy was more frequent in high Gleason sum carcinomas than in low. The incidence of aneuploidy in carcinomas with high Gleason grade (Gleason sum 8 to 10) was 77.8%. comparing to 33.3% in low Gleason grade (Gleason sum 2 to 4). When carcinomas classified according to both DNA ploidy and degree of glandular differentiation, then subgroups with the highest and lowest degree of malignant potential became apparent. None of diploid tumors with low Gleason grade (Gleason sum 2 to 4) formed metastasis, but 71.4% of aneuploidy tumors with high Gleason grade (Gleason sum 8 to 10) formed metastases. The influence of DNA ploidy on survival was examined with Kaplan-Meier method and the generalized Wilcoxon test. Overall, the patients with diploid tumor had a survival advantage over patients with aneuploid tumor (p<0.05). In patients with stage C and D, there was increasing tendency of survival in diploid group. In conclusion flow cytometric determination of DNA ploidy in prostatic adenocarcinoma is correlated strongly with clinical stage and Gleason sum and can be expected to be a valuable adjunct b clinical stage and histopathological grade in the assessment of malignant potential of prostatic adenocarcinoma.
Adenocarcinoma* ; Aneuploidy ; Diploidy ; DNA* ; Humans ; Incidence ; Neoplasm Metastasis ; Ploidies* ; Prognosis

BACKGROUND: Although postmenopausal estrogen replacement therapy is known to reduce cardiovascular mortality, the mechanism is not clear yet. Furthermore, the effect of estrogen on vascular tonus is reportedly variable according to the animal models, vascular beds and agonists used. MATERIALS AND METHOD: Bilateral ovariectomies were performed in 12 week-old, 18 spontaneously hypertensive rats (SHR) and 18 normotensive Wistar-Kyoto rats (WKY). Rats were divided into three groups according to the dose of 17beta-estradiol (E 2 ) pellets implanted subcutaneously two weeks after ovariectomy: control (no implantation), low-dose (0.5 mg) and high-dose (5 mg) E 2 replacement group. Two weeks after pellet implantation, organ bath experiments were performed using descending thoracic aortae. For endothelium-dependent relaxation, acetylcholine (10(-9) -3x10(-6) M) was cumulatively added into the vessels precontracted with 10(-7) M norepinephrine (NE). For vasoconstrictor responses, cumulative concentration-contraction curves were constructed in quiescent vessels using NE (10(-9) -10(-5) M), U46619 (10(-9) -3x10(-6) M), endothelin-1 (10(-10) -10(-7) M). In addition, contraction to angiotensin II (10(-7) M) was also obtained. Serum 17beta-estradiol levels were measured by radioimmunoassay. Blood pressure was measured by tail-cuff method in some SHRs before ovariectomy and after placebo/E 2 replacement. RESULTS: Endothelium-dependent relaxation to acetylcholine was impaired in WKY treated with 5 mg E 2 (pIC 50 : control vs 5mg E 2 : 7.75+/-0.13 vs 7.27+/-0.16: n=6: p<0.05). No significant effect was noted in SHR. Contraction to angiotensin II was inhibited by low-dose E 2 in WKY and high-dose E 2 in SHR (% of the contraction to 60 mM KCl: WKY: control vs 0.5 mg E 2 : 39+/-5 vs 25+/-2: SHR: control vs 5 mg E 2 : 34+/-4 vs 22+/-2: n=6 and p<0.05 in WKY and SHR). In contrast, NE-induced contraction was enhanced by E 2 replacement (both low- and high-dose) in WKY and SHR (WKY: control vs 0.5 mg E 2 vs 5 mg E 2 : AUC: 280+/-24 vs 387+/-26 vs 374+/-25: maximal contraction: 137+/-8 vs 166+/-8 vs 162+/-3: pD 2 : 7.63+/-0.11 vs 8.17+/-0.13 vs 8.13+/-0.13: SHR: control vs 0.5 mg E 2 vs 5 mg E 2 : AUC: 265+/-17 vs 349+/-16 vs 406+/-19: maximal contraction: 152+/-6 vs 181+/-9 vs 203+/-16: pD 2 : 7.45+/-0.13 vs 7.91+/-0.08 vs 8.04+/-0.04: n=6 and p<0.05 between control and treated groups in WKY and SHR for all parameters). Contraction to U46619 was enhanced by E 2 replacement in SHR (control vs 0.5 mg E 2 : AUC: 478+/-30 vs 574+/-23: maximal contraction: 181+/-9 vs 230+/-10: n=6: p<0.05 for both parameters). Maximal contractile response to endothelin-1 was also enhanced in SHR (control vs 0.5 mg E 2 vs 5 mg E 2 : maximal contraction: 165+/-7 vs 189+/-7 vs 199+/-8: n=6 and p<0.05 between control and treated groups) but not in WKY. Blood pressure was not different between placebo and E 2- treated SHR (171+/-2 vs 174+/-4 mmHg). CONCLUSION: In WKY, chronic high-dose estrogen replacement impairs endothelium-dependent relaxation to acetylcholine.: low-dose estrogen replacement does not affect endothelium-dependent relaxation in SHR and WKY. Estrogen replacement enhances the contraction to most of the contractile agonists tested except angiotensin II in both WKY and SHR. These results suggest that estrogen replacement affect the vascular tonus differently according to the vasoactive substances and/or hormones without significant effect on blood pressure.
15-Hydroxy-11 alpha,9 alpha-(epoxymethano)prosta-5,13-dienoic Acid ; Acetylcholine ; Angiotensin II ; Animals ; Aorta, Thoracic ; Area Under Curve ; Baths ; Blood Pressure ; Endothelin-1 ; Estrogen Replacement Therapy* ; Estrogens* ; Female ; Models, Animal ; Mortality ; Norepinephrine ; Ovariectomy ; Radioimmunoassay ; Rats ; Rats, Inbred SHR* ; Relaxation

Cardiac arrest associated with hyperkalemia during red blood cell transfusion is a rare but fatal complication. Herein, we report a case of transfusion-associated cardiac arrest following the initiation of extracorporeal membrane oxygenation support in a 9-month old infant. Her serum potassium level was increased to 9.0 mEq/L, soon after the newly primed circuit with pre-stored red blood cell (RBC) was started and followed by sudden cardiac arrest. Eventually, circulation was restored and the potassium level decreased to 5.1 mEq/L after 5 min. Extracorporeal membrane oxygenation (ECMO) priming is a relatively massive transfusion into a pediatric patient. Thus, to prevent cardiac arrest during blood-primed ECMO in neonates and infants, freshly irradiated and washed RBCs should be used when priming the ECMO circuit, to minimize the potassium concentration. Also, physicians should be aware of all possible complications associated with transfusions during ECMO.
Blood Transfusion ; Death, Sudden, Cardiac ; Erythrocyte Transfusion ; Erythrocytes ; Extracorporeal Membrane Oxygenation* ; Heart Arrest* ; Humans ; Hyperkalemia* ; Infant* ; Infant, Newborn ; Potassium