BACKGROUND: Endocrine pancreas tumor is a rare disease which incidence is less than 2% of all pancreatic tumors. But it comprises various types of tumor and usually secretes several hormones from one type of tumor although the patient with this tumor complains of sole symptom associated with only one hormone. The mechanism and clinical significance of multiple hormone secretion in the endocrine pancreas tumom are not yet clearly defined. METHODS: We analyzed retrospectively the clinicopathologic features of 20 cases which were operated at Seoul National University Hospital during the period between February 1989 and May 1998. RESULTS: The most common tumor was insulinoma (13 cases) and the second most common tumor was nonfunctioning tumor (6 cases). There was one case of somatostatinoma. Most of the patients with insulinoma complained of neuroglycopenic symptoms. There were 9 cases (45.0%) in which the tumors secreted more than two kinds of hormones, 7 cases in insulinoma, 2 cases in nonfunctioning tumors. Whether the tumor secreted multiple hormones was detected by the method of immunohistochemical staining. Though the tumors secreted more than two kinds of hormones, the patients with the tumors complained of symptoms which were associated with the cell type most strongly stained by immunohistochemical method. Whether or not the tumors secreted multiple hormones was not associated with the pathologic features such as tumor size, histologic patterns of the tumor, status of tumor cell differentiation and malignancy. CONCLUSION: From this results, we suggest that endocrine tumors of the pancreas secreted multiple hormones not by the mechanism of dedifferentiation from already differentiated endocrine cells but by the mechanism of neogenesis of multipotent islet stem cells. Since the relationship between the function of multiple hormone secretion in the endocrine pancreas tumors and islet stem cell would be significant, further study should be needed to find out the function of stem cells and application of stem cells to clinical use.
Cell Differentiation ; Endocrine Cells ; Humans ; Incidence ; Insulinoma ; Islets of Langerhans ; Pancreas* ; Rare Diseases ; Retrospective Studies ; Seoul ; Somatostatinoma ; Stem Cells

PURPOSE: Many inborn errors of metabolism can be completely cured with early detection and early treatment. This is why neonatal screening on inborn errors of metabolism is implemented worldwide. In this study, a cost-benefit analysis was performed on the neonatal screening of phenylketonuria and congenital hypothyroidism in Korea. METHODS: This study included 2,908,231 neonates who took the neonatal screening on phenylketonuria and congenital hypothyroidism in Korea from January 1991 to December 2003. From those neonates, the incidence rates of phenylketonuria and congenital hypothyroidism were measured. Furthermore, based on 495,000 babies born in 2002, were calculated and compared the total costs in case when neonatal screening on phenylketonuria and congenital hypothyroidism is implemented, and when not. RESULTS: If the neonatal screening on phenylketonuria and congenital hypothyroidism is implemented, benefits far exceed costs at a ratio of 1.77:1 in phenylketonuria, and 11.11:1 in congenital hypothyroidism. In terms of wons, the present neonatal screening on phenylketonuria and congenital hypothyroidism will gain us more than 29 billion wons every year. CONCLUSION: This study only concerns the monetary aspects of the neonatal screening. Therefore, the benefits of the neonatal screening is underestimated by ignoring precious but not measurable values such as quality of life. However, the present neonatal screening on phenylketonuria and congenital hypothyroidism is found to be beneficial and should continue for the good of the nation as well as that of the patients.
Congenital Hypothyroidism* ; Cost-Benefit Analysis* ; Humans ; Incidence ; Infant, Newborn ; Korea* ; Metabolism, Inborn Errors ; Neonatal Screening* ; Phenylketonurias* ; Quality of Life

BACKGROUND: Aside from its well known peripheral antihypertensive effects, calcium also lowers blood pressure, when administered into the cerebral ventricle. The present study was aimed to determine whether the central depressor response to calcium is mediated by a stimulation of endogenous L-arginine-nitric oxide (NO) pathway. METHODA: Mean arterial pressure (MAP) and heart rate (HR) were continuously recorded from the femoral artery in anesthetized rats. Administration of calcium was performed into the right lateral cerebral ventricle. The effects of N G-nitro-L-arginine methyl ester (L-NAME) on the cardiovascular response to calcium were examined. RESULTS: Intracerebroventricular (ICV) injection of calcium consistently produced a decrease in MAP and HR. The depressor and bradycardiac responses to calcium showed a dose-dependent fashion. Pretreatment with a calcium channel blocker, diltiazem (1 micromol, ICV), attenuated cardiovascular responses to calcium. ICV infusion (1 microl/min) of L-NAME (200 microgram/kg and 20 microgram/kg/min for 60 min) increased MAP without significant changes in HR. Chronic ingestion of L-NAME (5 mg/100 ml in drinking water, 4 weeks) also increased the systolic blood pressure as compared with control. The depressor effect of ICV calcium was significantly diminished in acute or chronic L-NAME treated rats. CONCLUSION: These findings suggest that the central depressor response to calcium, at least in part, is NO-dependent.
Animals ; Arterial Pressure ; Blood Pressure ; Calcium Channels ; Calcium* ; Cerebral Ventricles ; Diltiazem ; Drinking Water ; Eating ; Femoral Artery ; Heart Rate ; NG-Nitroarginine Methyl Ester ; Nitric Oxide* ; Rats

OBJECTIVE: The objective of this study was to determine the relationship between the fetal doppler flow velocimetry and birth weight in low risk pregnancy population. METHODS: From December 1995 to May 1996, We prospectively performed doppler study in 254 uncomplicated, term pregnant women, who visited Pohang Hospital, Dongguk University. Using pulsed color doppler, we measured umbilical artery RI, middle cerebral artery RI and middle cerebral-umbilical artery RI ratio within one week before delivery. RESULTS: The result was that low birth weight group (below 2500gm) had very significant lationship with umbilical artery RI(P<0.01), middle cerebral artery RI(P<0.05) and middle cerebral-umbilical artery RI ratio(P<0.05), but there was no significant relationship in these blood flow indices between normal birth weight group (2501gm- 3999gm) and macrosomia group (above 4000gm). CONCLUSIONS: We concluded that application of doppler ultrasonopaphy in the assessment of fetal weight is somewhat helpful for identification of low birth weight, not for macrosomia.
Arteries ; Birth Weight ; Female ; Fetal Weight* ; Gyeongsangbuk-do ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Middle Cerebral Artery ; Pregnancy ; Pregnant Women ; Prospective Studies ; Rheology ; Ultrasonography* ; Umbilical Arteries

Systemic lupus erythematosus(SLE), a rheumatic disease of unknown causes, is characterized by autoantibodies directed against self-antigens, resulting in inflammatory damages to target organs including kidney, blood-forming cells, and central nervous system. The prevalence rates are higher in Native Americans, Asians, Latin Americans and black people. A female to male ratio of approximately 2 : 1 occurs before puberty, and 4 : 1 after puberty. Children of SLE most frequently present with fever, fatigue, arthralgia or arthritis, and rashes. The diagnosis is confirmed by clinical and also laboratory manifestations satisfying at least 4 out of 11 criteria. A central nervous system is not rarely involved in children of SLE with the prevalence rate of 23-44%. However, cerebral infarcts are not a common phenomenon and can be seen only for 6% of children with SLE. There have been no cases reported in Korea. This is why we present a case of basal ganglia infarct associated with SLE in a 19-month old girl. We report this case with a brief review of related literature.
Adolescent ; Arthralgia ; Arthritis ; Asian Continental Ancestry Group ; Autoantibodies ; Autoantigens ; Basal Ganglia* ; Central Nervous System ; Child ; Diagnosis ; Exanthema ; Fatigue ; Female ; Fever ; Humans ; Indians, North American ; Infant ; Kidney ; Korea ; Lupus Erythematosus, Systemic ; Male ; Prevalence ; Puberty ; Rheumatic Diseases

Holoprosencephaly is a rare and complex malformation affecting the cleavage of the developing forebrain and is usually associated with defects of the mid Face. We have experienced a case of holoprosencephaly, diagnosed prenatally by ultrasound examination at 31 weeks of pregnancy in a 31-year-old primigravida woman. This case is characterized by holoprosencephaly, cleft palate, cleft lip, left renal aplasia and right renal hypertrophy. The chromosomal study showed a deletion of the long arm of chromosome 7, 46, XX, del(7)(q32), We report with a terminal deletion of chromosome 7q associated with atypical clinical picture and holoprosencephaly.
Adult ; Arm ; Chromosomes, Human, Pair 7 ; Cleft Lip ; Cleft Palate ; Female ; Holoprosencephaly* ; Humans ; Hypertrophy ; Pregnancy ; Prosencephalon ; Ultrasonography

Deranged expression of cell cycle modulators has been reported to contribute to the development and progression of hepatocellular carcinoma (HCC). However, their expression patterns remain poorly understood in hepatitis B virus (HBV)-related HCC, which constitutes about 65-70% of HCC in Korea. The aims of this study were to evaluate the expressions of G1-S modulators in HBV-related HCCs and dysplastic nodules (DNs), and to correlate with the histopathologic features of HCCs. Immunohistochemical expressions of cyclin D1, cyclin E, p53, p27, p21, p16, Rb, and PCNA proteins were investigated in 80 HCCs and 22 DNs. Cyclin D1 overexpression showed positive relationships with advanced tumor stage, poor differentiation, larger tumor size, microvascular invasion, intrahepatic meta-stasis, no tumor capsule formation, infiltrative growth, aberrant p53 expression, and high PCNA labeling index (LI) of HCC (p<0.05). Aberrant p53 expression showed positive relationship with poor differentiation of HCC (p<0.01). Expression of cyclin D1 or p53 was not observed in DNs. The p27 LI and p16 LI were lower in HCCs with intrahepatic metastasis (p<0.05). Cyclin D1 overexpression and aberrant p53 expression could be associated with the progression of HBV-related HCC, and might have a less crucial role in the DN-HCC sequence. In addition, elevated expression of p27 and p16 proteins might have inhibitory action to the intrahepatic metastasis of HBV-related HCC.
Adult ; Aged ; Carcinoma, Hepatocellular/chemistry/etiology/*pathology ; Cyclin D1/*analysis ; Female ; G1 Phase ; Hepatitis B/*complications ; Human ; Immunohistochemistry ; Liver Neoplasms/chemistry/etiology/*pathology ; Male ; Microfilament Proteins/analysis ; Middle Age ; Precancerous Conditions/*virology ; Proliferating Cell Nuclear Antigen/analysis ; Protein p16/analysis ; Protein p53/*analysis ; Retinoblastoma Protein/analysis ; S Phase

BACKGROUND: An increase of the extracellular K+concentrations up to about 8 mM in the isolated vessels causes relaxation in pre-contracted state. In order to elucidate the mechanisms of K+induced relaxation and compare with that of 2-kidney, 1 clip (2K1C) renal hypertensive rats, we recorded aortic vascular tension using an organ bath study. METHOD: 2K1C hypertension was made by clipping the left renal artery and age-matched control rats received a sham treatment. Thoracic aortic rings were mounted in tissue baths for measurement of isometric contractile force. RESULTS: Exposure to K+(from 2 to 8 mM) relaxed a phenylephrine (2 x 10-6 M)-induced contraction in K+free Krebs-Ringer solution, dose-dependently. Ouabain (10-5 M) enhanced the K+induced relaxation in above 2 mM K+ The K+induced relaxation was still induced in endothelium-denuded condition. Incubation with the K+channel blockers such as tetraethylammonium (TEA, 1 mM), glibenclamide (10-5 M), 4-aminopyridine (3 mM), barium (5 mM) and cesium (2 mM) did not affect on the K+induced relaxation. In renal hypertensive rats, the K+induced relaxation was markedly suppressed and ouabain enhanced it. CONCLUSIONS: These results suggest that the K+induced relaxation in aorta be mediated by Na-pump independent mechanisms, and the decrease of the K+induced relaxation in the renal hypertensive rats may be a possible mechanism of hypertension.
4-Aminopyridine ; Animals ; Aorta ; Barium ; Baths ; Cesium ; Glyburide ; Hypertension ; Muscle, Smooth* ; Ouabain ; Phenylephrine ; Placebos ; Rats* ; Relaxation ; Renal Artery ; Tetraethylammonium ; Vasodilation*

BACKGROUND: An increase of the extracellular K+concentrations up to about 8 mM in the isolated vessels causes relaxation in pre-contracted state. In order to elucidate the mechanisms of K+induced relaxation and compare with that of 2-kidney, 1 clip (2K1C) renal hypertensive rats, we recorded aortic vascular tension using an organ bath study. METHOD: 2K1C hypertension was made by clipping the left renal artery and age-matched control rats received a sham treatment. Thoracic aortic rings were mounted in tissue baths for measurement of isometric contractile force. RESULTS: Exposure to K+(from 2 to 8 mM) relaxed a phenylephrine (2 x 10-6 M)-induced contraction in K+free Krebs-Ringer solution, dose-dependently. Ouabain (10-5 M) enhanced the K+induced relaxation in above 2 mM K+ The K+induced relaxation was still induced in endothelium-denuded condition. Incubation with the K+channel blockers such as tetraethylammonium (TEA, 1 mM), glibenclamide (10-5 M), 4-aminopyridine (3 mM), barium (5 mM) and cesium (2 mM) did not affect on the K+induced relaxation. In renal hypertensive rats, the K+induced relaxation was markedly suppressed and ouabain enhanced it. CONCLUSIONS: These results suggest that the K+induced relaxation in aorta be mediated by Na-pump independent mechanisms, and the decrease of the K+induced relaxation in the renal hypertensive rats may be a possible mechanism of hypertension.
4-Aminopyridine ; Animals ; Aorta ; Barium ; Baths ; Cesium ; Glyburide ; Hypertension ; Muscle, Smooth* ; Ouabain ; Phenylephrine ; Placebos ; Rats* ; Relaxation ; Renal Artery ; Tetraethylammonium ; Vasodilation*

BACKGROUND: Calcium plays a key role in vascular contraction and regulates receptor sensitivity to certain neurotransmitters. Calcium channel blockers are useful in the treatment of both clinical and experimental hypertension. The present study was designed to examine whether there is an alteration of the activity of calcium channels in association with the development of hypertension. METHODS: Deoxycorticosterone acetate(DOCA)-salt hypertension was made by subcutaneous implantation of DOCA(200mg/kg)strip plus saline drinking(1%) and 2-kidney, 1 clip(2KIC)hypertension by clipping the left renal artery with a silver clip(internal gap of 0.2mm). They were used 4 weeks later. Age-matched normal rats served as a control. Mean arterial pressure(MAP) and heart rate(HR) were continuously recorded from the right femoral artery. The drugs were administered intravenously. RESULTS: Vehicle alone was without effect on MAP or HR. In normotensive rats, nifedipine infusion(5 and 10ug/kg/min)caused a dose-dependent decrease in MAP without significant changes in HR, while Bay k 8644(Bay K, 5 and 10 ug/kg/min) increased MAP transiently. Both the depressor response to nifedipine and the pressor response to Bay k were more marked in DOCA-salt hypetensive rats than in normotensive rats. The maximal changes in MAP indced by nifedipine(5 and 50 ug/kg) or Bay K(5 and 50 ug/kg) were also enhanced in 2KIC hypertensive rats as compared with control rats. CONCLUSION: These results indicate that calcium channel inhibitors and activators can affect on the regulation of blood pressure in an opposite fashion. It is also suggested that the activity of calcium channels might be altered in the developement of experimental hypertension.
3-Pyridinecarboxylic acid, 1,4-dihydro-2,6-dimethyl-5-nitro-4-(2-(trifluoromethyl)phenyl)-, Methyl ester* ; Animals ; Bays* ; Blood Pressure ; Calcium ; Calcium Channel Blockers ; Calcium Channels ; Desoxycorticosterone ; Femoral Artery ; Heart ; Hypertension ; Neurotransmitter Agents ; Nifedipine* ; Rats* ; Renal Artery ; Silver