No abstract available.
Germ Cells* ; Neoplasms, Germ Cell and Embryonal*

Crouzon's disease, first described by Crouzon in 1912, is characterized by craniosynostosis, exorbitism and midface hypoplasia. As well, it has been known to be transmitted as an autosomal dominant trait. Clinically, Crouzon's disease not only has aesthetic problems, but also many functional disabilities, such as increased intracranial pressure, hydrocephalus, visual disturbance. difficulty in nasal breathing and malocclusion. The surgical correction of Crouzon's disease includes: 1) frontal bone advancement and release of the craniosynostosis; 2) correction of the midface retrusion; and 3) other ancillary procedures for better aesthetic results. The authors performed Le Fort III advancement for the correction of midface retrusion in 5 cases of Crouzon's disease. These advancements were performed at about the age of 15 in consideration of facial bone growth disturbance, psychosocial effects, and the recurrence of class III malocclusion after operation. The average advancement of the midface was 9. 2 mm. We experienced favorable aesthetic and functional results without severe complication in all cases or relapse of the advanced segment.
Craniofacial Dysostosis* ; Craniosynostoses ; Facial Bones ; Frontal Bone ; Hydrocephalus ; Intracranial Pressure ; Malocclusion ; Recurrence ; Respiration

Germ cell tumors(GCT) are not infrequently encounted tumor in pediartic age group. In order to study the clinical behaviors of the disease, we reviewed 29 cases of GCT diagnosed and treated at Kosin Medical Center in the period of 7 years form Jan. 1984 to Dec. 1991 and the results are summarized as follows: 1) The age distribution is the highest between 10 and 15 years(45%) and girls have 1.9 times higher frequency than that of boys. 2) The most frequent primary site of GCT was ovary(15). The next common primary site was testis(5), pineal body(4), sacrococcygeal region(2), retroperitoneum(1), soft palate(1), anterior mediastinum(1) in descending order of frequency. 3) In the pathological analysis of tumors, the most frequent type was mature teratoma(12). The next common type was endodermal sinus tumor(6), immature teratoma(5), dysgerminoma(3), mixed type(2), and choriocarcinoma(1)in descending order of frequency. 4) All the 5 cases of EST have elevated serum levels of alpha-fetoprotein. Two of the three dysgerminoma, one of the two mixed type tumors both of which have germinoma components, and one choriocarcinoma have elevated serum levels of beta-HCG above the age related physiological levels. 5) Among 17 cases of malignant GCT, 9 cases have metastatic areas such as regional lymph nodes, scrotum, pleura, retroperitoneum, omentum and lumbar spine. 6) All except on case of immature teratoma in the anterior mediastinum underwent surgery. Chemotherapy was given to 12 of 17 malignant GCT and radiotherapy was given to 4 of 17 malignant GCT. 7) Among the 4 expired cases within 12 months of follow up, three were originated from pineal body, which had been underwent simple V-P shunt or incomplete resection, and one case was metastatic immature teratoma of anterior mediastinum to the pleura which had not been undergone surgery. Finally, in review of literature and or cases, the survival of properly managed GCT except some of those difficult surgical access is relatively good. To continue the survival of children of GCT to be impoved, we should use all the treatment modalit properly with more positive outlook about the disease.
Age Distribution ; alpha-Fetoproteins ; Child* ; Choriocarcinoma ; Drug Therapy ; Dysgerminoma ; Endoderm ; Female ; Follow-Up Studies ; Germ Cells* ; Germinoma ; Humans ; Lymph Nodes ; Mediastinum ; Neoplasms, Germ Cell and Embryonal* ; Omentum ; Pineal Gland ; Pleura ; Pregnancy ; Radiotherapy ; Scrotum ; Spine ; Teratoma

No abstract available.
Bone and Bones* ; Dental Implants*

Wolf-Hirschhorn syndrome (WHS) is a malformation associated with a hemizygous deletion of the distal short arm of chromosome 4. Herein we report a fetal autopsy case of WHS. A male fetus was therapeutically aborted at 17(+0) weeks gestational age, due to complex anomaly and intrauterine growth retardation, which were found in prenatal ultrasonography. His birth weight was 65 g. Mild craniofacial dysmorphism, club feet, bilateral renal hypoplasia, edematous neck, and left diaphragmatic hernia of Bochdalek were found on gross examination. On GTG-banding, the fetus revealed 46,XY,add(4p) karyotype and the mother revealed 46,XX,t(4;18)(p16;q21.1), with normal karyotype of the father. Array comparative genomic hybridization performed on the autopsied lung tissue revealed loss of 4p16.2-->4pter and gain of 18q21.1-->18qter, suggesting 46,XY,der(4)t(4;18)(p16.2;q21.1)mat of fetal karyotype. This suggested deletion of 4p, compatible with WHS inherited from the mal-segregation of a maternal translocation t(4;18)(p16.2;21.1). Therefore, our fetus was both genotypically and phenotypically compatible with WHS.
Arm ; Autopsy ; Birth Weight ; Chromosomes, Human, Pair 4 ; Comparative Genomic Hybridization ; Fathers ; Fetal Growth Retardation ; Fetus ; Foot ; Gestational Age ; Hernia, Diaphragmatic ; Humans ; Karyotype ; Karyotyping ; Lung ; Male ; Mothers ; Neck ; Ultrasonography, Prenatal ; Wolf-Hirschhorn Syndrome

No abstract available.
Appendicitis* ; Child* ; Humans

No abstract available.
Hemoperitoneum*

Recent studies suggest that coronary endothelial dysfunction in atherosclerosis may contribute to the predisposition both for coronary spasm as well as for thrombus formation. The integrity of at least one aspect of endothelial function can be assessed by the intracoronary administration of acetylcholine. Eleven(13.4%, mean age 48+13, male 11) out of 82 consecutive patients with acute myocardial infarction showed angiographically normal or near normal coronary arteries at 7~10 in-hospital days. Ten(91%) had history of heavy smoking and 7 patients(64%) had no history of previous angina. Anterior myocardial infarction was in 8 and inferior in 3 patients. After intracoronary administration of acetylcholine(incremental dose of 20, 50ug for the right and 20, 50, 100ug for the left), 9(82) of 11 infarct related arteries showed total or subtotal occlusion, but only in 3(17%) out of 18 non-infarct related arteries of 10 patients. Multivessel spasm was noticed in 3. Ergonovine test(graded doses of 0.05, 0.1 and 0.2 mg intravenously) was performed 30 minutes after completion of acetylcholine test in 5 patients. In 1 patients, acetylcholine test was positive and but ergonovine test was negative. Conclusion : 1) Eleven(13.4%) out of 82 patients with acute myocardial infarction had angiographically normal coronary arteries. 2) Ach positive responses of infarct-related arteries are more frequent(82%) than that of non-infarct related arteries(17%), suggesting that the infarction might be related to coronary artery spasm. 3) Positive Ach responses of non-infarct related arteries occurred in 30% of Patients. 4) Ach and Erg induced vasospasm developed at the same site in 5, which suggests that local hyperreactivity might be related to spasm.
Acetylcholine* ; Arteries ; Atherosclerosis ; Coronary Vessels* ; Ergonovine ; Humans ; Infarction ; Male ; Myocardial Infarction* ; Smoke ; Smoking ; Spasm* ; Thrombosis

Chronic iron overload is associated with life-threatening complications, such as cardiomyopathy, liver cirrhosis, diabetes, hypothyroidism, hypoparathyroidism and hypogonadism. We experienced a case of secondary hemosiderosis with hepatic fibrosis in a 8-year-old boy who had been transfused 56 pints of packed red blood cells for a supportive therapy of acute myelogenous leukemia for a 27-month period. Intramuscular injection of Desferroxamine (40mg/kg/ day) was done for 9 months, however, there was no sign of improvement in hepatic size, consistency, and in liver biopsy findings. Except for mild thrmobocytopenia which seems to be the result of hypersplenism, the boy remained in remission for 3 2/3 years. We think, the prompt use of Desferroxamine therapy may be needed to avoid iron overload, if patients recieved multiple transfusions and high serum ferritin level.
Biopsy ; Blood Transfusion ; Cardiomyopathies ; Child ; Deferoxamine ; Erythrocytes ; Ferritins ; Fibrosis* ; Hemosiderosis* ; Humans ; Hypersplenism ; Hypogonadism ; Hypoparathyroidism ; Hypothyroidism ; Injections, Intramuscular ; Iron Overload ; Leukemia, Myeloid, Acute* ; Liver ; Liver Cirrhosis ; Male

No abstract available.
Polyps*