BACKGROUND: Childhood myelodysplastic syndrome(MDS) is a heterogenous disease complex which has characteristics of cytopenia in one or more hemopoietic cell lines in peripheral blood and of dysmorphisms of hemopoietic precursors in bone marrow, but quite different from adult one. We experienced 22 patients with childhood myelodysplastic syndrome during 9 years from 1987 to 1995 in the Department of Pediatrics, Inje University College of Medicine, Pusan Paik Hospital, Pusan, Korea and performed a clinical study about them to analyze the clinical and hematological features and treatment outcome retrospectively. METHOD: We analyzed their clinical features including age and sex distribution, chief complaints and physical findings on first admission, distribution of subtypes, hematologic features including initial hemoglobin, total WBC and platelet count, and treatment outcome including leukmic transformation and prognostic scores. RESULTS: 1) Of the total 22 patients, 10 were in 0-4 years of age on first admission, 8 in 5-9, and 4 above 10 years of age. The male : female sex ratio was 2.1 : 1. 2) Of the 22 patients, chief complaints on first admission were bleeding tendency in 9 patients(40.9%), pallor in 8(36.4%) and fever in 3(13.6%), in order, and physical findings on first admission were purpura or ecchymoses in 17(77.3%), anemia in 15(68.2%) and hepatosplenomegaly in 8(36.4%), in order. 3) Hematologic findings on first admission were as follows : hemoglobin levels were below 3 g/dl in 1 patient(4.5%), 3-6 g/dl in 10(45.5%), and 6-9 g/dl in 11(50.0%). Initial WBC counts were below 5,000/mm3 in 13 patients(59.1%), 5,000-10,000/mm3 in 5(22.7%) and above 10,000/mm3 in 4(18.2%). Initial platelet counts were below 20,000/mm3 in 10(45.5%), 20,000-50,000/mm3 in 5(22.7%), 50,000-100,000/mm3 in 5(22.7%) and above 100,000/mm3 in 2(9.1%). 4) Of the 22 patients, 12 patients(54.6%) were RA type, 1(4.6%) RAS, and 3(13.6%) RAEB, RAEB-T and JCML types, respectively. 5) According to prognostic scores by Mufti et al(1986), none were in 'good' group, 17 patients(89.5%) in 'intermediate' group with 39.5 months of mean duration of survival(range 4-95 months) and 2(10.5%) in 'poor' group with 18 months of mean duration of surviral(range 17-19) until the last follow-up. However, the subtypes and clinical status seemed not to be related to the prognostic scores. 6) Sixteen patients were treated with low dose cytosine arabinoside(10 mg/m2/12hrs), of whom 7 patients gained long-standing event-free survival, whose treatment regimen was changed to oral 6-TG about 2 years later. All 3 of JCML were treated with A-Triple-V regimen, one of whom was died of sepsis, one was transformed into AML and died of sepsis, while the remained one gained long-standig event-free survival (62 months). 7) Leukemic transformation into AML occured in 7 patients(RA 1, RAEB 2, RAEB-T 3, JCML 1), 6 of whom were dead, while one gained long-standing event-free survival of 34 months. CONCLUSION: We concluded that RA was the most dominant type among our patients, and the frequency to transform into AML was 31,8%, and 31.9% of the patients had long-term survival, and that reliability of prognostic scoring system by Mufti et al(1986) was not high.
Adult ; Anemia ; Anemia, Refractory, with Excess of Blasts ; Bone Marrow ; Busan ; Cell Line ; Cytosine ; Disease-Free Survival ; Ecchymosis ; Female ; Fever ; Follow-Up Studies ; Hemorrhage ; Humans ; Korea ; Male ; Myelodysplastic Syndromes* ; Pallor ; Pediatrics ; Platelet Count ; Purpura ; Retrospective Studies ; Sepsis ; Sex Distribution ; Sex Ratio ; Treatment Outcome

Cytologic findings from five cases with variable types of Hodgkin' disease were reviewed with special emphasis on the Reed-Sternberg (R-S) cells and their variants. Typical R-S and Hodgkin's cells were mono- or binucleated, and nuclei had rounded smooth contour. Acidophilic prominent nucleoli with perinucleolar halo were conspicuous. In comparison to typical Reed-Sternberg cells, L & H (lymphocytic and histiocytic) cells in the lymphocyte predominant type tended to show pop-corn like irregular nuclear contour and to lack the prominent nucleoli. Lacunar cells in the nodular sclerosis type had multilobated nuclei with prominent acidophilic nucleoli. There was no prominent perinucleolar halo in L & H and lacunar cells. In conjuction with the number of Reed-Sternberg cells and back ground findings observed on the smears, the characteristic features of R-S cells and their variants allowed to make typing of Hodgkin's disease.
Hodgkin Disease* ; Lymphocytes ; Reed-Sternberg Cells* ; Sclerosis

Gastric glomus tumor is an uncommon benign, submucosal neoplasm and does not require radical surgical procedure. Because there are no specific clinical or radiologic features associated with the glomus tumor, it can be recognized only by its histologic characteristics. We report a 30-year-old woman who had 10 years history of epigastric hunger pain. Radiologically, a gastric submucosal tumor was discovered, which was suggestive of leiomyoma. Gastric antrectomy was performed. The tumor cells showed immunohistochemical and ultrastructural evidence of smooth muscle differentiation.
Female ; Humans

Iron deficient state occurs commonly in the athlets, and the cause may be inadequate iron intake, loss of iron from sweat, blood loss of gastrointestinal tract, and hematuria. The age of the athletes ranged from 11 to 17 years old. I messured red blood cell count, hemoglobin, hematocrit, and related hematologic factors in the 32 adolescent female athletes and 21 female controls. a hematologic comparison was perfomed between the athletes and controls. The results obtained were as follows: 1) A value of hemoglobin were 11.6+/-1.4g/dl in the athletes and 12:4+/-0.8g/dl in controls (p<0.05). 2) Hematocrits were 34.1+/-3.4% in the athletes and 37.4+/-2.3% in controls (p<0.05). 3)A value of red bolld cell distribution width values were 13.9+/-2.1% in the athletes and 12.2+/-1.1% in controls (p<0.05). 4) Serum iron was 87.7+/-30.3ug/dl in the athletes and 121.8+/-39.0ug/dl in controls (p<0.001). 5) Total iron binding capacity was 445:6+/-31.8ug/dl in the athletes and 384:6+/-54.2ug/dl in controls (p<0.001). 6) Mean transferrin saturation values were 19.7+/-6.9% in the athletes and 32.5+/-10.8% in control (p<0.001). 7) Ferritin was 14.5+/-10.0ng/ml in the athletes and 33.9+/-13.9ng/ml in controls (p<0.001). 8) The duration of exercise was 4.8+/-2.5 years in the stage III iron deficiency, whil 3.0+/-2.0 years in normal group in iron deficient state (p<0.05).
Adolescent* ; Athletes* ; Erythrocyte Count ; Female* ; Ferritins ; Gastrointestinal Tract ; Hematocrit ; Hematuria ; Humans ; Iron* ; Sweat ; Transferrin

No accepted standard currently exists to classify asphyxia and define its subtypes. Sauvageau and Boghossian proposed an asphyxia classification system in 2010 that divided asphyxia into suffocation, strangulation, mechanical asphyxia, and drowning. Here, we present a modification of this classification system. We propose to classify asphyxia into four main categories: suffocation, strangulation, mechanical asphyxia, and complicated asphyxia. Suffocation includes smothering and choking as well as confined spaces, entrapment, and vitiated atmosphere. Strangulation is subdivided into hanging, ligature strangulation, manual strangulation, and other unspecified strangulation. Mechanical asphyxia includes positional and traumatic asphyxia. Finally, complicated asphyxia is defined as cases with two or more identifiable mechanisms of asphyxia. In this study, we review autopsy cases from 2012 diagnosed as asphyxia and classify them according to our proposed asphyxia classification system. In 24.7% of cases, the age range was 40-49 years, and 51.9% were men. The most common method of asphyxia was hanging (245 cases, 55.1%), followed by ligature or manual strangulation (53 cases, 11.9%). Most hangings were suicides; smothering, ligature, and manual strangulation were usually homicides. Eighteen cases were complicated asphyxia. This classification provides a simplified, unified, and useful tool to classify and understand deaths due to asphyxia.
Airway Obstruction ; Asphyxia* ; Atmosphere ; Autopsy* ; Classification* ; Confined Spaces ; Drowning ; Homicide ; Humans ; Korea* ; Ligation ; Male ; Suicide

Aneurysm of the internal carotid artery is a rare disease and is known to be associated with congenital arterial anomalies such as neurofibromatosis type I (NF-I). NF-I is an autosomal dominant neurocutaneous disorder characterized by a variety of manifestations that involve the central and peripheral nervous systems, skin, vascular system, and skeleton. In particular, the involvement of vascular abnormalities in NF-I is well known. Any vessel may be affected by this condition, although the renal artery is most frequently involved. The vascular abnormality can be occlusive or an aneurysmal degenerative change. Therefore, symptomatic presentations might assume an indolent pathophysiologic course such as hypertension, or manifest as a catastrophic event such as arterial rupture that could result in sudden death. We report a rare autopsy case of an aneurysmal rupture of the internal carotid artery in a woman with suspected NF-I, who collapsed in her home.
Aneurysm ; Autopsy ; Carotid Artery, Internal ; Death, Sudden ; Female ; Glycosaminoglycans ; Humans ; Hypertension ; Neurocutaneous Syndromes ; Neurofibromatoses ; Neurofibromatosis 1 ; Peripheral Nervous System ; Rare Diseases ; Renal Artery ; Rupture ; Skeleton ; Skin

Polypoid cystitis is a rare entity and, to our knowledge, has not been described in the radiologic literature.It is frequently found in patients with indwelling catheters and is characterized by inflammation and edema of thelamina propria. We encountered a case of papillary cystitis which on a sonogram showed a solid mass withinhomogeneous internal echopattern ; on CT, a poorly-defined inhomogeneous, dense, soft-tissue density mass withenhancement and perivesical extension ; and on T2 weighted MR images an inter mediate SI mass.
Carcinoma, Transitional Cell* ; Catheters, Indwelling ; Cystitis* ; Edema ; Humans ; Inflammation

Complications after a median sternotomy incision, which is used currently in most open heart surgery, are serious, although it is infrequent. Reconstruction of the sternal defect resulting from dehiscence of median sternotomy is still big challenge to the most plastic surgeons. Since vascularized greater omentum was transposed to eliminate mediastinal wound problems, many vascularized regional muscle flaps became mainstay in reconstruction of median sternotomy wound. We treated 13 patients with median sternotomy dehiscence between October of 1993 and March of 1998. In two patients, the wound problems were so confined to superficial tissue that debrided and closed primarily. Eleven patients with deep wound infection were managed with vigorous debridement of all necrotic tissues and resultant defects were covered with regional muscle flaps: rectus myocutaneous flap(3) and bilateral pectoralis advancement flap(8). We used the pectoralis major advancement flaps without counter incision at humeral insertion site and the dissections were limited only medial to the anterior axillary line to preserve the axillary fold. In five patients with larger defects, we elevated muscle and cutaneous flaps separately to make these flaps more mobile. Large portion of two rectus abdominis flaps could not survive, whereas pectoralis advancement flaps had mo special wound problems. Only one patient developed fistula due to remained wire, regardless to flap surgery.
Debridement ; Fistula ; Humans ; Omentum ; Rectus Abdominis ; Sternotomy* ; Thoracic Surgery ; Wound Infection ; Wounds and Injuries

The intravenous administration of midazolam is widely used as sedative premedication for upper gastrointestinal endoscopy. We performed a study to evaluate the effectiveness and safety of midazoiam as premedication for upper gastrointestinal endoscopy. Between June 1995 and October 1995, 112 patients of diagnostic esophago-gastroduodenoseopy were enrolled in the study. The patients were recieved a bolus midazolam 0.~03mg/kg or placebo, followed by typical anesthesia. The blood pressure, pulse and oximeter values were monitored. The systolic blood pressure and heart rate were increased during endoscopy in compared with before premedication, and normalized immediately. There was no significant change of arterial oxygen saturation. Midazolam induced amnesia completely in 49.2% and partially in 27.2%, and all patients were recovered completely in 1 hour. The tolerance score is higher in the midazolam group as compared with the placebo group(p<0.05), and midazolam group would accept the same sedation for repeated endoscopies(p<0.01). We conclude that midazolam has beneficial effects as premedication for upper gastrointastinal endoscopy without significant altteration in cardiopulmonary parameters. This suggest that midazolam may be used more frequently as premedication, especially in the cases of repeated endoscopy.
Administration, Intravenous ; Amnesia ; Anesthesia ; Blood Pressure ; Endoscopy ; Endoscopy, Digestive System ; Endoscopy, Gastrointestinal* ; Heart Rate ; Humans ; Midazolam* ; Oxygen ; Premedication*

No abstract available.
Animals ; Brain* ; Inositol* ; Phospholipases* ; Rats*