No abstract available.
Carotid Artery, Internal ; Carotid Artery, Internal, Dissection ; Osteogenesis Imperfecta

A pancreatico-pleural fistula (PPF), caused by rupture of a pancreatic pseudocyststectomy or obstruction of the pancreatic duct, is a rare condition. A 48-year-old man with chronic alcoholism was admitted with a massive pleural effusion. Pleural fluid studies revealed elevated amylase and lipase. A PPF complicated by a ruptured pancreatic pseudocyststectomy was diagnosed by computerized tomography scan. Although the symptoms improved with conservative management, (chest tube drainage, NPO, total parenteral nutrition, and a pancreatic secretion inhibitor), a distal pancreatectomy, including a pseudocystectomy and thoracotomy, were performed for an increasing size of the hemorrhagic pancreatic pseudocyststectomy and a recurrent hemorrhagic pleural effusion. There were no post-operative complications and the patient was discharged on post-operative day 27.
Alcoholism ; Amylases ; Drainage ; Fistula ; Humans ; Lipase ; Middle Aged ; Pancreatectomy ; Pancreatic Cyst ; Pancreatic Ducts ; Parenteral Nutrition, Total ; Pleural Effusion ; Rupture ; Thoracotomy

A left atrial appendage aneurysm is a very rare medical condition which can develop by an inflammatory reaction or a degenerative change. If there is no accompanying anomaly, a left atrial appendage is considered a congenital disease. The majority of left atrial appendage aneurysms are detected incidentally because they usually do not cause any symptoms. Surgery is indicated, even for asymptomatic patients, because of the risk of life-threatening complications, such as atrial fibrillation, supraventricular tachycardia, systemic embolization, and cardiac arrest. Left atrial appendage aneurysms are usually treated by a median sternotomy with extracorporeal circulation, especially if the aneurysm has a broad base or contains a thrombus, but can treated by thoracotomy without extracorporeal circulation. We report a case of a successfully treated left atrial appendage aneurysm that was misdiagnosed as a partial pericardial defect without extracorporeal circulation in a 13-year old child.
Aneurysm ; Atrial Appendage ; Atrial Fibrillation ; Extracorporeal Circulation ; Heart Arrest ; Heart Atria ; Humans ; Sternotomy ; Tachycardia, Supraventricular ; Thoracotomy ; Thrombosis

BACKGROUND: Descending necrotizing mediastinitis (DNM) is a life-threatening cervico-mediastinal infection extending from the oropharynx or periodontal space. We reviewed clinical outcomes of DNM patients that underwent surgical management. MATERIAL AND METHOD: We analyzed the demographic and surgical data from 8 patients (6 males and 2 females) that underwent surgical management for DNM between August 2003 and August 2007. RESULT: The mean age was 56.6+/-12.3 (34~72) years. Types of DNM were I (n=2), IIA (n=1), and IIB (n=5), based on the classification system of Endo et al. Four patients were septic at the time of operation. The infectious organism was identified in three cases and turned out to be Streptococcus. ICU stay was 24.3+/-17.9 (3~58) days, and hospital stay was 49.1+/-33.8 (20~125) days. There were two deaths (25%), both of which were due to multi-organ failure. CONCLUSION: Despite aggressive surgical drainage and appropriate medical management, DNM still had a high mortality rate. Early diagnosis and prompt surgical intervention are key to DNM management. In addition, transcervical drainage should be used in limited disease only.
Drainage ; Early Diagnosis ; Humans ; Length of Stay ; Male ; Mediastinitis ; Necrosis ; Oropharynx ; Streptococcus

BACKGROUND: The histomorphological properties of the left anterior desecending artery (LAD), the left internal thoracic arteries (LITA), the radial arteries (RA) and the intercostal arteries (ICA) were studied for their use as a conduit for coronary artery bypass grafting (CABG), and we compared them with each other. MATERIAL AND METHOD: All the vessels were harvested from nineteen cadavers (17 males and 2 females). The mid-portion of the LAD, the mid-portion of the LITA, the distal RA and the mid-portion of the 5th ICA were obtained. All of them were stained with hematoxylin-eosin and with Van Gieson's elastin stain. The morphological characteristics were examined and the thicknesses of the intima and media (I/M ratio: the intima to media ratio) were compared using one-way ANOVA tests. RESULT: The mean age of the cadavers was 61.5+/-9.6 years. The LITA and ICA were elastic arteries, and the LAD and RA were muscular arteries. The I/M ratio showed statistically significant differences: 0.07+/-0.03 in the LITA, 0.16+/-0.11 in the ICA, 0.45+/-0.29 in the RA and 0.93+/-0.52 in the LAD, respectively. CONCLUSION: This study showed that the degrees of intimal hyperplasia of the CA and the various conduits for CABG were different significantly. The ICA was found to have relatively favorable characteristics as a coronary bypass conduit, but its suitability for clinical use is a challenging issue.
Arteries ; Cadaver ; Coronary Artery Bypass ; Coronary Vessels ; Elastin ; Humans ; Hyperplasia ; Male ; Mammary Arteries ; Radial Artery ; Transplants

BACKGROUND: The aim of this study was to investigate the prevalence, clinical and laboratory findings of hereditary hemolytic anemia (HHA) in Korea from 1997 to 2006 and to develop the appropriate diagnostic approach for HHA. METHODS: By the use of questionnaires, information on the clinical and laboratory findings ofHHA diagnosed from 1997 to 2006 in Korea was collected and analyzed retrospectively. A total of 431 cases were enrolled in this study from 46 departments of 35 hospitals. RESULTS: The overall frequency of HHA did not change through the 10-year period for pediatrics but did show an increasing tendency for internal medicine. The overall male to female sex ratio did not show sex predominance (1.17:1), but a significant male predominance with a ratio of 1.49:1 was seen for pediatrics while a significant female predominance with a ratio of 1:1.97 was seen forinternal medicine. Of the total cases, 74.2% (282/431) were diagnosed before the age of 15 years. The etiologies of HHA were classified as red cell membrane defects, hemoglobinopathies, red cell enzyme deficiencies and unknown causes. There were 382 cases (88.6%) of red cell membrane defects with 376 cases (87.2%) of hereditary spherocytosis and 6 cases (1.4%) of hereditary elliptocytosis, 20 cases (4.6%) of hemoglobinopathies with 18 cases (4.2%) of beta-thalassemia, a case (0.2%) of alpha-thalassemia and a case (0.2%) of Hemoglobin Madrid, 7 cases (1.6%) of red cell enzyme deficiencies with 5 cases (1.2%) of glucose-6- phosphate dehydrogenase (G-6-PD) deficiency, a case (0.2%) of pyruvate kinase (PK) deficiency and a case (0.2%) of enolase deficiency, and 22 cases (5.1%) of unknown causes. The most common chief complaint in pediatric patients was pallor and that in adult patients was jaundice. In the red cell membrane defect group of patients, the level of hemoglobin was significantly higher than in adult patients. The mean corpuscular volume, mean corpuscular hemoglobin, corrected reticulocyte count, total and indirect bilirubin level and lactate dehydrogenase levels in the hemoglobinopathy group of patients were significantly lower than the values in the red cell membrane defect group of patients. The mean concentration of G-6-PD was 0.8+/-0.7U/1012RBC in the G-6-PD deficient patients, PK was 1.7U/1010 RBC in the PK deficient patient, and the level of enolase was 0.04U/g of Hb in the enolase deficient patient. CONCLUSION: The most prevalent cause of HHA in Korea during 1997 to 2006 was hereditary spherocytosis, but HHA by other causes such as hemoglobinopathy and red cell enzyme deficiency gradually increased with the development of molecular diagnostic methods and increasing general interest. However, the etiologies of HHA need to be pursued further in 5.1% of the patients. An systematic standard diagnostic approach is needed in a nationwide prospective study for correct diagnoses and appropriate management of HHA.
Adult ; alpha-Thalassemia ; Anemia, Hemolytic, Congenital* ; beta-Thalassemia ; Bilirubin ; Cell Membrane ; Diagnosis ; Elliptocytosis, Hereditary ; Erythrocyte Indices ; Female ; Hemoglobinopathies ; Humans ; Internal Medicine ; Jaundice ; Korea* ; L-Lactate Dehydrogenase ; Male ; Oxidoreductases ; Pallor ; Pathology, Molecular ; Pediatrics ; Phosphopyruvate Hydratase ; Prevalence ; Pyruvate Kinase ; Reticulocyte Count ; Retrospective Studies* ; Sex Ratio ; Surveys and Questionnaires

BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is one of the most important armamentarium against various hematologic malignancies or some solid tumors. We investigated the number of patients who might need transplants and compared with that of actual transplants to conceptualize current status and circumstances of HSCTs in Korean children. METHODS: Questionnaires were sent to Korean Society of Hematopoietic Stem Cell Transplantation (KSHSCT) members who were taking care of children with malignancies or hematologic diseases. Almost all of the newly diagnosed patients between Jan, 1st and Dec, 31st, 2003 were enrolled in the study. RESULTS: Seven hundred forty eight children (male to female ratio = 1.4:1) were enrolled. The median age was 6.1 years old (8 days~28.8 years old). Malignant diseases consisted of 695 cases (92.9%), and among them almost half were hematologic malignancies. The participating members speculated that HSCTs should be indicated in 285 children (38.1%) which included 209 allogeneic, and 76 autologous transplants. In reality, however, allogeneic HSCTs were performed only in 140 children (67.0%) with the median interval of 5.9 month, and autologous transplants in 44 children (57.9%) with 8.3 month. In autologous setting, all the patients received peripheral blood stem cells (PBSCs), whereas bone marrow (61%), cord blood (34%), and PBSC (5%) were used in allogeneic HSCTs. Donor types were as follows: unrelated donor (37%), cord blood (34%), sibling donor (25%), and family (4%). The reasons for not performing HSCTs were unfavorable disease status or death, no availability of suitable donor, economical situation, and refusal by parental preferences. Under the strict insurance regulations, many transplants were not covered by insurance. More autologous transplants were performed without insurance coverage than allogeneic HSCTs (P=0.013). Those cases were advanced cases and HLA mismatch transplants for allogeneic setting, and relatively rare diseases still awaiting favorable results of transplants for autologous setting. CONCLUSION: HSCTs are essential part of treatment strategies for children with various diseases. Unfortunately, however, a third of patients who were in need of transplants did not receive HSCTs due to various reasons. It is necessary to expand unrelated donor pool or cord blood banks for the cases lacking HLA-identical sibling donors. Also medical insurances should cover HSCTs for rare diseases as well as for less favorable but novel situations where there are no suitable alternatives.
Autografts ; Bone Marrow ; Child* ; Disulfiram ; Female ; Fetal Blood ; Hematologic Diseases ; Hematologic Neoplasms ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Insurance ; Insurance Coverage ; Parents ; Rare Diseases ; Siblings ; Social Control, Formal ; Stem Cells ; Tissue Donors ; Unrelated Donors ; Surveys and Questionnaires

We report a 32-year-old woman who developed an altered consciousness and quadriparesis three days after ingesting an ergot alkaloid derivative to suppress lactation. Brain MRI showed extensive cerebral infarction in the posterior circulation territory with multifocal vasoconstriction in the intracranial vessels including both the middle cerebral arteries. Extensive studies for stroke etiology are negative. Follow-up MRA and serial TCD studies revealed that reVersible vasospasm was the mechanism. This case is additional evidence that ergot derivatives may lead to postpartum cerebral angiopathy.
Adult ; Brain ; Cerebral Infarction ; Consciousness ; Female ; Follow-Up Studies ; Humans ; Lactation ; Magnetic Resonance Imaging ; Middle Cerebral Artery ; Postpartum Period* ; Quadriplegia ; Stroke ; Vasoconstriction ; Vasospasm, Intracranial

The interstitial cells of Cajal (ICCs) are the pacemaker cells in gastrointestinal tract and generate electrical rhythmicity in gastrointestinal muscles. Therefore, ICC may be modulated by endogenous agents such as neurotransmitter, hormones, and prostaglandins (PGs). In the present study, we investigated the effects of prostaglandins, especially PGE2, on pacemaker currents in cultured ICCs from murine small intestine by using whole-cell patch clamp techniques. ICCs generated spontaneous slow waves under voltage-clamp conditions and showed a mean amplitude of -452+/-39 pA and frequency of 18+/-2 cycles/min (n=6). Treatments of the cells with PGE2 (1muM) decreased both the frequency and amplitude of the pacemaker currents and increased the resting currents in the outward direction. PGE2 had only inhibitory effects on pacemaker currents and this inhibitory effect was dose-dependent. For characterization of specific membrane EP receptor subtypes, involved in the effects of PGE2 on pacemaker currents in ICCs, EP receptor agonists were used: Butaprost (1muM), EP2 receptor agonist, reduced the spontaneous inward current frequency and amplitude in cultured ICCs (n=5). However sulprostone (1muM), a mixed EP1 and EP3 agonist, had no effects on the frequency, amplitude and resting currents of pacemaker currents (n=5). SQ-22536 (an inhibitor of adenylate cyclase; 100muM) and ODQ (an inhibitor of guanylate cyclase; 100muM) had no effects on PGE2 actions of pacemaker currents. These observations indicate that PGE2 alter directly the pacemaker currents in ICCs, and that the PGE2 receptor subtypes involved are the EP2 receptor, independent of cyclic AMP- and GMP-dependent pathway.
Adenylyl Cyclases ; Dinoprostone* ; Gastrointestinal Tract ; Guanylate Cyclase ; Interstitial Cells of Cajal* ; Intestine, Small* ; Membranes ; Muscles ; Neurotransmitter Agents ; Patch-Clamp Techniques ; Periodicity ; Prostaglandins

PURPOSE: Childhood acute immune thrombocytopenic purpura (ITP) is a benign hematologic disease. Therapy does not affect the natural history of the illness. We evaluated the clinical and laboratory findings, treatment and prognosis of childhood acute ITP in Korea through a retrospective multicenter study. METHODS: We analyzed retrospectively the data of 1, 829 children with acute ITP through survey of 33 hospitals among 43 hospitals in Korea from Sep. 1992 to Aug. 2001. RESULTS: Male to female ratio was 1.3: 1 and the median age at the diagnosis of ITP was 2.9 (0.1 17) years. Median duration of follow up was 6 months. One hundred and forty nine cases of the total 1, 829 patients (8.1%) received no treatment. The initial median platelet count of the non-treated group was 42, 500/mm3. Among the 861 cases who were followed up over 6 months, 315 cases (36.6%) progressed into chronic ITP. Associated with this high rate of chronicity of childhood acute ITP patients in Korea, we must consider the fact that acute ITP patients with fast improvement in the first episode tend not to follow up. Considering that fact, the rate of chronicity becomes 17.2% of the 1, 829 acute ITP patients. The treated group used many kinds of treatment methods. Intravenous immunoglobulins (IVIG) with or without prednisolone (PD) (67.5%) were the most commonly used regimens. In the group treated with IVIG alone, the platelet count began to rise above 50, 000/mm3 at 2.6 days, 100, 000/mm3 at 3.7 days and 150, 000/mm3 at 4.9 days. Four hundred and twenty two cases of the 1, 686 (25.0%) cases followed up after first episode of ITP relapsed. The relapse rate was significantly higher in older patients and in girls than in younger patients and in boys (P< 0.05). The chronicity of ITP statistically increased with age (P< 0.05) and that was the only valuable factor. CONCLUSION: Despite the fact that childhood acute ITP is a pretty common disaese, there is no agreement on the best treatment method for this disease. The establishment of Korean treatment guideline of childhood acute ITP, based on an analysis of multicenters, seems to be needed.
Child ; Diagnosis ; Female ; Follow-Up Studies ; Hematologic Diseases ; Humans ; Immunoglobulins, Intravenous ; Korea* ; Male ; Natural History ; Platelet Count ; Prednisolone ; Prognosis ; Purpura, Thrombocytopenic, Idiopathic* ; Recurrence ; Retrospective Studies