No abstract available.

Citrullinemia is an inborn error of urea cycle metabolism caused by deficiency of arginosuccinate synthetase. It is characterized by hyperammonemia and high citrulline level in serum, CSF and urine. The clinical symptoms include vomiting, lethargy, seizure, coma and ultimately death if hyperammonemia is not controlled. We report a case of 9- day old male with citrullinemia who was initially treated with sodium benzoate during acute stage followed by gradual weaning to discontinuation. Hyperammonemia was well controlled by low protein milk diet and arginine.
Arginine* ; Citrulline ; Citrullinemia* ; Coma ; Diet* ; Humans ; Hyperammonemia ; Lethargy ; Ligases ; Male ; Metabolism ; Milk ; Seizures ; Sodium Benzoate ; Urea ; Vomiting ; Weaning

PURPOSE: To compare the effect of breast milk containing fortifier with preterm formula on bone mineral metabolism in premature infants, we evaluated the state of bone mineralization, biochemical alteration and the frequency of rickets during the first five months of life. METHODS: Fourteen fortified breast milk-fed infants and eleven preterm formula-fed infants who were born at Il-Sin Christian Hospital from August, 1996 through July, 1997, were studied. The breast milk-fed group received human milk fortifier. The intake of Ca, P, Mg, protein, and fat was calculated at one month of age. Birth weight and weight at one, two and five months of age were measured. Serum Ca, P, alkaline phosphatase and 25-hydroxyvitamine D were measured at one month of age, and the wrist received X-ray examinations monthly. Total body bone mineral content was measured by Dual energy X-ray absorptiometry (Lunar WI) at two and five months of age. RESULTS: The two groups were similar in birth weight, gestational age, and weight at one, two and five months of age. Enteral Ca, P, protein, Mg, and fat intake, and urinary excretion of Ca, P were similar among the two groups. Serum Ca, P, ALP and 25-hydroxyvitamin D were not different. Occurrence of rickets and bone mineral content were similar among the two groups. CONCLUSION: Fortified breast milk-fed infants and preterm formula-fed infants showed no difference in total body bone mineral content and occurrence of rickets. Ultimately, we could enhance the advantage of breast milk fed to premature infants by adding fortifier.
Absorptiometry, Photon ; Alkaline Phosphatase ; Birth Weight ; Bone Density ; Breast ; Calcification, Physiologic ; Gestational Age ; Humans* ; Infant ; Infant, Newborn ; Infant, Premature ; Metabolism* ; Milk, Human* ; Rickets* ; Wrist

PURPOSE: Persistent pulmonary hypertension (PPHN) of the newborn has been treated with some vasodilators including tolazoline. But these drugs have many side effects, especially systemic hypotension . To investigate the usefulness of the nitroglycerin as a specific vasodilator with few side effects, this study was done. METHODS: Nitroglycerin was administered within 1st one day after birth in 8 newborn infants who were diagnosed as PPHN by echocardiography. They were born at Il Sin hospital from March 1994 to March 1996. Nitroglycerin was started as 2microgram/kg/min and its maximum dose was 6microgram/kg/min. Muscle relaxants and inotropic drugs were used together in all cases. Arterial blood gas analysis, systemic blood pressure, heart rate, renal function and electrolyte were checked in all patients. Alveolar-arterial oxygen difference (AaDO2) and oxygenation index (OI) were measured. RESULTS: 6 cases (75%) survived but 2 cases expired due to air leak.1) Basal mean AaDO2 was 631.4+/-21.7mmHg. It decreased to 493.9+/-1453.1 mmHg at 10hr after loading infusion and to 373.6+/-217.7mmHg at 48hr (P<0.05). 2) Basal mean OI was 35.1+/-15.7 and it decreased significantly to 12.6+/-14.8 (P<0.05) at 10hr. 3) There was no significant hypotension in systolic, diastolic and mean blood pressure during treatment of nitroglycerin. 4) There was no significant change in renal function, serum electrolyte and heart rate during treatment of nitroglycerin. CONCLUSIONS: Nitroglycerin produced systemic venodilatation and pulmonary arterial dilatation at the dose that produce only minimal systemic arterial dilatation. Nitroglycerin is an effective and safe drug in the treatment of PPHN.
Blood Gas Analysis ; Blood Pressure ; Dilatation ; Echocardiography ; Heart Rate ; Humans ; Hypertension, Pulmonary* ; Hypotension ; Infant, Newborn* ; Nitroglycerin* ; Oxygen ; Parturition ; Tolazoline ; Vasodilator Agents

Aneurysms arising from ICA bifucation are relatively rare. But they are paticulary difficult to treat surgically because of perforating arteries surrounding and adherent to the aneurysm. In an effort to determine their best management policy, and to improve their surgical outcome, a retrospective clinical analysis of 30 patients who underwent direct surgery from ICA bifucation aneurysm between January, 1984 and December, 1994 was performed. In the same period, total 990 patients with intracranial aneurysms were admitted in our department. The result of carotid bifucation aneurysm surgery was worse than the total surgical result. Motality and morbidity rates were 3/30(10%), 5/30(16%). Surgical complications in this group of aneurysms usually came from injury of perforating arteries, and the causes of death were intraoperative premature rupture and vasospasm. The surgical treatment of aneurysms of internal carotid bifucation is discussed, emphasizing the importance of avoiding damage to perforating vessels and premature rupture. A classification of these aneurysms, according to angiographic and anatomical considerations, is proposed. We suggest that four types of aneurysmal orientation at the bifucation of the internal carotid artery be distinguished:either projecting superiorly, anteriorly, inferiorly, or posteriorly. Posterior orientation was found in 13 cases(43.3%). Superior orientation was found in 11 cases(36.7%), inferior orientation was found in 4 cases(13.3%), and anterior orientation was found in 2 cases(6.7%). From an analysis of these cases and a review of the literature, some characteristics of these aneurysms and their surgical strategies are described.
Aneurysm* ; Arteries ; Carotid Artery, Internal ; Cause of Death ; Classification ; Humans ; Intracranial Aneurysm ; Retrospective Studies ; Rupture

PURPOSE: To evaluate the causative factors in the catastrophic failure of LCP in the proximal humerus fracture. MATERIALS AND METHODS: Six patients (6 cases) were collected between October 2003 and July 2005. The mean age was 55.6 years (range: 38~70). The cause of injury was road traffic accident in four, fall down in one and slip down in one. According to the Neer classification, four were 2 part fractures, each one in 3 part fracture and 4 part fracture. RESULTS: Fixation failure occurred due to back-out of the plate and screw in five and plate breakage in one. Analysis of the preoperative radiographs revealed medial cortical defect in all and no bone graft and tension band wiring in the greater tuberosity fragment were carried out. Postoperative radiographs showed the anatomical reduction in three and non-anatomical in three. CONCLUSION: Non-anatomical reduction, insufficient medial bony buttress, inadequate screw length to the head and the neglect for the greater tuberosity fragment were the contributing factors to the failure of LCP. Knowledge of these factors will enable the surgeon to avoid failure of the LCP. Augmentation fixation and bone graft procedures with careful preoperative planning are necessary for successful fixation of LCP.
Accidents, Traffic ; Classification ; Head ; Humans ; Humerus* ; Transplants

Concurrent cerebral and coronary artery embolization is a theoretically possible, but extremely rare complication of an atrial myxoma. We present a paitent with a left atrial mass (a probable myxoma) who presented with concurrent cerebral and myocardial infarctions due to emboli of tumor origin. An 84-year-old woman presented with an acute cerebral infarction of the middle cerebral artery territory. Several hours after admission, she complained of chest pain consistent with a myocardial infarction. Transthoracic and transesophageal echocardiographic studies revealed the presence of a large, mobile, heteroechoic mass with a few daughter nodules in the left atrium, compatible with a myxoma. Coronary angiography disclosed subtotal occlusion of the ramus intermedius branch and visible tumor vascularization adjacent to the right coronary artery. With medical treatment, including anticoagulation, the patient was stabilized and had an uneventful clinical course for the ensuing 6 months since discharge.
Aged, 80 and over ; Cerebral Infarction ; Chest Pain ; Coronary Angiography ; Coronary Vessels ; Female ; Heart Atria ; Humans ; Middle Cerebral Artery ; Myocardial Infarction ; Myxoma ; Nuclear Family

PURPOSE: The critical pathway (CP) is to standardize the clinical practice of specialists working to optimize care. The objective of this study was to develop a critical pathway for the surgical treatment of patients with colorectal or gastric cancer and to evaluate the results of the CP. METHODS: Twenty-one patients with colorectal cancer, who were managed according to the CP between August 2005 and November 2005, were compared with 18 patients for whom this pathway had not been used between June 2004 and September 2004. Forty-eight patients with gastic cancer, who were managed according to the CP between June 2005 and September 2005, were compared with 49 patients for whom this pathway had not been used daring the same period in 2004. The length of stay and the cost per patients were compared between the CP group and the non-CP group. RESULTS: For patients with colorectal cancer, the postoperative hospital length of stay in the CP group was significantly shorter (9.0 vs. 12.3 days, P<0.001), but for patients with gastric caner, there was no difference (10.6 vs. 11.4, P=0.134). The mean hospital charges were won5,037,816 and won5,263,508 for colorectal cancer and for gastric cancer, respectively, and won4,808,602 and won4,674,329, for the CP and the non-CP groups, respectively, but these differences were not significant. CONSLUSIONS: The critical pathway in colorectal and stomach surgery decreased the length of stay and might regulate hospital charges. Such a pathway could be easily designed and implemented at hospitals and could standardize clinical practice.
Colorectal Neoplasms ; Critical Pathways* ; Hospital Charges ; Humans ; Length of Stay ; Specialization ; Stomach ; Stomach Neoplasms*

No abstract available.
Carcinoma, Hepatocellular* ; Pregnancy*

PURPOSE: HER2 is a 185-kDa transmembrane protein, which shares a considerable homology with the epidermal growth factor receptor. The over-expression of HER2 has been reported to be associated with a poor clinical outcome in breast cancer. In clinical practice and research studies, immunohistochemistry (IHC) and fluorescence in-situ hybridization (FISH) have commonly been used for the detection of HER2. The aim of this study was to evaluate the prognostic significance of HER2 according to the results of IHC and FISH for HER2. METHODS: IHC and FISH were performed on the same breast cancer specimens from 388 Korean patients, with a mean follow-up duration of 59.8 months, with survival analyses were made according to the results of the HER2 detection methods; A0485 Ab (DAKO, Denmark) and the HER2 test kit (DAKO HerceptTest(TM), Denmark) for IHC, and the HER2 FISH kit (Vysis Inc., Downers Grove, Ill) for FISH were used. RESULTS: The IHC showed HER2 over-expression rates of 34.8% and 26.8% by the A0485 antibody and HercepTest, respectively. The rate of HER2 amplification by FISH in the same specimen was 25.8%. HER2 was confirmed as an independent prognostic factor by multivariate analyses regardless of the detection methods. In the survival analyses, according to the IHC results for HER2, only the 3+ scoring group in the positive results was associated with a poor survival. However, the positive group in the FISH test revealed a significantly worse survival than the FISH-negative group. CONCLUSION: For the prediction of survival of patients with breast cancer, the FISH test would be more useful than IHC, especially in the 2+ scoring IHC cases.
Breast Neoplasms* ; Breast* ; Fluorescence ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Multivariate Analysis ; Prognosis ; Receptor, Epidermal Growth Factor ; Survival Analysis*