PURPOSE: We report the results of a policy of primary surgical closure of large ventricular septal defects(VSDs) applied over the past 3 years to all symptomatic infants regardless of patient weight, location or number of VSDs. To analyze the early postoperative mortality and other surgical complications in young infants, comparison of preoperative and outcome variables were made between infants weighing 6 kg or less and those weighing mort than 6 kg at the time of operation. METHODS: Forty-eight infants met criteria for inclusion in the study, and were divided into two groups based on body weight: group 1 infants weighed 6 kg or less(n=24), and group 2 infants weighed more then 6 kg(n=24). Both groups had similar variation in ventricular septal defect location and the ratio of the flow. resistance. RESULTS: The major associated cardiac defects were more in group 1. The overall mortality rate of this study was 4%(2/48): Two early deaths occurred in only group 1(8%). Both infants had a doubly committed subarterial defect and mild degree of coarctation of aorta (one was a preductal type). One received a coatctation-repari. There was o only one patch dehiscence(in group 1). No surviving patients required a second ventricular septal defect opetation, and the majority no longer received anticongestive therapies. CONCLUSIONS: These results indicate that primary surgical closure of large ventricular septal defects can be performed in small infants with no difference in mortality or serious complication rates compared with larger infants. Protracted medical efforts to achieve larger size before primary repair and palliative pulmonary artery banding are not necessary.
Aortic Coarctation ; Body Weight ; Heart Septal Defects, Ventricular* ; Humans ; Infant* ; Mortality ; Pulmonary Artery

Acute necrotizing pancreatitis is a disease with high morbidity and mortality despite the progress made in intensive care. Gas gangrene of the pancreas, usually caused by anaerobe infection, is an extremely rare, but severe form of acute necrotizing pancreatitis. Several severity-of-illness classifications for acute pancreatitis are used to identify patients at risk for complications. Pancreatic necrosis is diagnosed radiographically by dynamic intravenous contrast-enhanced computed tomography (CT) of the abdomen. Early CT in patients with suspected necrotizing pancreatitis contributes to early intervention and many advantageously enhance survival. We report here on two cases of necrotizing pancreatitis with gas gangrene in 49-year-old male patient and 86-year-old female patient.
Abdomen ; Aged, 80 and over ; Classification ; Critical Care ; Early Intervention (Education) ; Female ; Gas Gangrene* ; Humans ; Male ; Middle Aged ; Mortality ; Necrosis ; Pancreas ; Pancreatitis* ; Pancreatitis, Acute Necrotizing

We herein describe a 70-year-old woman who presented with respiratory failure due to extensive lung adenocarcinoma. Despite advanced disease, care in the intensive care unit with ventilator support was performed because she was a newly diagnosed patient and was considered to have the potential to recover after cancer treatment. Because prompt control of the cancer was needed to treat the respiratory failure, empirical treatment with an oral epidermal growth factor receptor (EGFR) tyrosine kinase inhibitor was initiated before confirmation of EGFR-mutant adenocarcinoma, and the patient was successfully treated. Later, EGFR-mutant adenocarcinoma was confirmed.
Adenocarcinoma* ; Aged ; Female ; Humans ; Intensive Care Units ; Lung* ; Protein-Tyrosine Kinases ; Receptor, Epidermal Growth Factor ; Respiration, Artificial ; Respiratory Insufficiency* ; Ventilators, Mechanical ; Erlotinib Hydrochloride

BACKGROUND: Left ventricular hypertrophy is common in chronic renal failure patients and may contribute increased risk of cardiovascular morbidity and mortality. We evaluated the left ventricular morphology and function in renal transplant recipients to find the relationship between hemodynamic changes and morphologic and functional improvement after transplantation. METHODS: Serial echocardiographic evaluations were performed in 27 adults(20 men and 7 women) at the time of transplantaion and posttransplantation 1 month and 4 months. The average duration of hemodialysis was 16+/-24 months(mean+/-S.D.). RESULTS: At the time of transplantation, the hematocrit level was 21+/-6% and posttransplantation 1 month and 4 months, that was increased to 39+/-5% and 42+/-7%, respectively(p<0.001). Left ventricular mass index by echocardiography was decreased significantly from 246+/-87g/m2(pre-KT) to 169+/-38g/m2(post-KT 1 month) and 153+/-40g/m2(post-KT 4 months), respectively (p<0.001). Interventricular septal thickness and left ventricular posterior wall thickness were decreased significantly after 4 months of transplantation. Left ventricular systolic and diastolic dimensions were also decreased significantly after 1 month and 4 months of transplantation. Left ventricular volumes and cardiac output were also decreased significantly. But A/E ratio, ejection fraction and fractional shortening did not change significantly. CONCLUSION: These findings showed that pretransplant high output state was resolved radipidly(within 1 month) but the diastolic function did not improved after transplantation 1 month and 4 months.
Cardiac Output ; Echocardiography* ; Hematocrit ; Hemodynamics ; Humans ; Hypertrophy, Left Ventricular ; Kidney Failure, Chronic ; Kidney Transplantation ; Male ; Mortality ; Renal Dialysis ; Transplantation*

Familial combined hyperlipidemia is one af the manogenic disorders frequently found in humans and is seen in 0.5~2% of the general populatian, accounting for at least 10% of persons with pemature atlmmcletusis. The distinguishing feature of familial combined hyperlipidemia, in camparison with other single-gene abnarmalities of lipoprotein metabolism, is that not all affected members have the same plasma lipid phenotype; some individuals have an elevation of cholesterol concentration alane(type IIa lipoprotein pattern), while some athers have an elevation of triglyceride concentration alone(type IV pattem), and still others have elevations of both values(type IIb pattem). In any one persan, the lipid phenotype can change as a result of dietary or drug treatment. Familial combined hyperlipidemia should be suspected in those subjects with moderate hypertriglyceridemia and/or moderate hypercholestaolemia (lipoprotein types IIa, Ilb, IV), especially when premature coronary heart disease is evident in the family histary. Low plasma HDL-cholesterol, obesity, insulin resistance and hyperuricemia are often . Family members affected by familial combined hyperlipidemia should be identified and be treated, since tbe condition is associated with premature caronary heart diasease. We have found one family of familial combined hyperlipidemia with one member(case 1) associated with insulin resistance, hyperuricemia and gout, and another member(case 2) associated with diabetes mellitus and infertiTity.
Cholesterol ; Coronary Disease ; Diabetes Mellitus ; Gout ; Heart ; Humans ; Hyperlipidemia, Familial Combined* ; Hypertriglyceridemia ; Hyperuricemia ; Insulin Resistance ; Lipoproteins ; Metabolism ; Obesity ; Phenotype ; Plasma ; Triglycerides

We describe a unique patient with progressive external ophthalmoplegia, intestinal pseudo-obstruction, and neurogenic bladder. Genetic study in this patient shows point mutation at T8356C, the locus known as that of myoclonic epilepsy with ragged-red fibers. To the best of our knowledge, this is the first report of a mitochondrial syndrome consisting of intestinal pseudo-obstruction, neurogenic bladder, and progressive external ophthalmoplegia, point mutation at T8356C. We suggest that this could comprise a new mitochondrial disease rather than a new variant of mitochondrial neurogastrointestinal encephalomyopathy.
Humans ; Intestinal Pseudo-Obstruction* ; MERRF Syndrome ; Mitochondrial Diseases* ; Ophthalmoplegia, Chronic Progressive External ; Point Mutation* ; Urinary Bladder, Neurogenic*

Hypoglycemia due to non-islet cell tumor is usually associated with hypersecretion of big insulin-like growth factor II (IGF-II). This big IGF-II cannot form ternary IGF complex, and is biologically more active in peripheral tissue, inducing increased glucose utilization and hypoglycemia. A 57-year-old man developed severe hypoglycemia due to hepatocellular carcinoma. To control hypoglycemia, the patient required continuous glucose infusion. The circulating levels of cortisol and free T4 were in the normal range. The plasma levels of insulin, C-peptide, IGF-I, IGF binding protein-3 (IGFBP-3), and total IGF-II levels were decreased. Radioimmunoassay of IGF-II revealed that big IGF-II immunoreactivity markedly increased compared to that of normal control. In this patient, it was strongly suggested that big IGF-II might be a cause of severe intractable hypoglycemia.
C-Peptide ; Carcinoma, Hepatocellular ; Glucose ; Humans ; Hydrocortisone ; Hypoglycemia* ; Insulin ; Insulin-Like Growth Factor I ; Insulin-Like Growth Factor II* ; Middle Aged ; Plasma ; Radioimmunoassay ; Reference Values

We report here on a case of mucinous adenocarcinoma that probably originated in the renal pelvis of a horseshoe kidney. A 61-year-old woman presented with a palpable mass in the left upper quadrant of the abdomen, and this mass had been present for several months. Computed tomography (CT) revealed a left renal pelvic tumor in the horseshoe kidney. Grossly, a 10x9x8 cm unilocular cystic mass filled with chocolate colored mucinous fluid was seen. A connection between the cystic mass and the renal pelvis was demonstrated on retrograde pyelography. Microscopically, the cyst contained anaplastic columnar mucosecretory epithelial cells. Some atypical cell clusters were freely floating in the mucinous lakes. The histopathological findings were consistent with mucinous adenocarcinoma. In addition, glandular metaplasia was noted in the cystic wall. Immunohistochemical assessment of the pelvic adenocarcinoma revealed the positive expressions of carcinoembryonic antigen (CEA) and cytokeratin 20 (CK20) and a weak positive expression of cytokeratin 7 (CK7).
Female ; Humans ; Cysts ; Adenocarcinoma

The study was undertaken for the clinical evaluation and statistical analysis on the 222 patients with ectopic pregnancy who had been adbitted and treated at the Mokpo St.Columban's Hospital from Jan. 1, 1993 to Dec. 31, 1995. The results were obtained as follows. 1. Hospital incidence of ectopic pregnancy wasd 1 in 45 deliveries(222/10,078). 2. Ectopic pregnancy was found to occur most frequently in the age group, ranging from 31 to 35 years(36.9%). 3. Nullipara was 26.5% and woman who had experienced artificial abortion was 69.8%. 4. Hemoglobin value over 10.0 gm/dL was in 78.8% and below 8.9 gm/dL in 4.1%. 5. Initial systolic blood pressure rise above 100 mmHg was in 75.0%, the mean value was 104.5mmHg. 6. The clinical manifestations were appeared in 64.4% from the last menstrual peroid to the next 5~8 weeks. 7. Total amount of intraperitoneal hemorrhage between 100~1,000 ml was in 62.1%, above the 1,000 ml was in 29.3% and less than 100 ml was in 8.6%. 8. Ectopic pregnancy was implanted follopain tuve in 95.0% ovary in 4.5% and cervix in 0.5%. Among tubal pregnancies, ampulla portion was involved in 76.6%, interstitial portion in 3.1%, isthmic portion in 12.2% and fimbrial portion in 3.1%. 9. In the past history, the laparoscopic tubal ligation was in 18%, peritonitis was in 1.8%, appendectomy was in 7.2% and cesarean section was in 8.6%. 10. Culdocentesis was positive in 70.0% and urinary HCG test was positive in 90%. 11. 77.8% of total cases was treated by salpingectomy, 14.9% by salpingoophorectomy and 1.4% by hysterectomy. 12. Of 222 total cases, no death occurred.
Appendectomy ; Blood Pressure ; Cervix Uteri ; Cesarean Section ; Female ; Hemorrhage ; Humans ; Hysterectomy ; Incidence ; Jeollanam-do ; Ovary ; Peritonitis ; Pregnancy ; Pregnancy, Ectopic* ; Pregnancy, Tubal ; Salpingectomy ; Sterilization, Tubal

The study was undertaken for the clinical evaluation and statistical analysis on the 222 patients with ectopic pregnancy who had been adbitted and treated at the Mokpo St.Columban's Hospital from Jan. 1, 1993 to Dec. 31, 1995. The results were obtained as follows. 1. Hospital incidence of ectopic pregnancy wasd 1 in 45 deliveries(222/10,078). 2. Ectopic pregnancy was found to occur most frequently in the age group, ranging from 31 to 35 years(36.9%). 3. Nullipara was 26.5% and woman who had experienced artificial abortion was 69.8%. 4. Hemoglobin value over 10.0 gm/dL was in 78.8% and below 8.9 gm/dL in 4.1%. 5. Initial systolic blood pressure rise above 100 mmHg was in 75.0%, the mean value was 104.5mmHg. 6. The clinical manifestations were appeared in 64.4% from the last menstrual peroid to the next 5~8 weeks. 7. Total amount of intraperitoneal hemorrhage between 100~1,000 ml was in 62.1%, above the 1,000 ml was in 29.3% and less than 100 ml was in 8.6%. 8. Ectopic pregnancy was implanted follopain tuve in 95.0% ovary in 4.5% and cervix in 0.5%. Among tubal pregnancies, ampulla portion was involved in 76.6%, interstitial portion in 3.1%, isthmic portion in 12.2% and fimbrial portion in 3.1%. 9. In the past history, the laparoscopic tubal ligation was in 18%, peritonitis was in 1.8%, appendectomy was in 7.2% and cesarean section was in 8.6%. 10. Culdocentesis was positive in 70.0% and urinary HCG test was positive in 90%. 11. 77.8% of total cases was treated by salpingectomy, 14.9% by salpingoophorectomy and 1.4% by hysterectomy. 12. Of 222 total cases, no death occurred.
Appendectomy ; Blood Pressure ; Cervix Uteri ; Cesarean Section ; Female ; Hemorrhage ; Humans ; Hysterectomy ; Incidence ; Jeollanam-do ; Ovary ; Peritonitis ; Pregnancy ; Pregnancy, Ectopic* ; Pregnancy, Tubal ; Salpingectomy ; Sterilization, Tubal