No abstract available.
Classification* ; Myelodysplastic Syndromes*

Three autopsy cases of arthrogryposis multiplex congenita are studied. They were two deadborns and one neonatal death. All of them had characteristic abnormalities involving multiple joints. Neither primary myopathy nor abnormalities of anterior horn cells of the spinal cord were detected in our cases. However, two cases had minor central nervous system anomalies. All four cases showed pulmonary hypoplasia of varying degree. Two of three cases showed facial dysmorphism such as micrognathia and low set ears, and one showed cleft lip and palate. Ventricular septal defect, umbilical hernia and ureteral anomalies were also associated.
Infant, Newborn ; Humans

Body stalk anomaly represents an extreme maldevelopment of embryonic body folding and is characterized by absence of the umbilicus and umbilical cord. The failure of complete obliteration of the extraembryonic coelom is responsible for the absence of the umbilical cord formation and the wide-based insertio of the amnioperitoneal membrane onto the placental chorionic plate. We have analyzed 10 autopsy cases of various midline anomalies of the body that could best be classified into body stalk anomaly. All cases were either stillborns or dead immediately after birth. The pregnancy was interrupted due to this anomaly in 6 cases, and their gestational ages varied from 17 weeks to 37 weeks. The affected fetuses were characterized bt absent or vestigial umbilical cord, and ruptured amnion with direct amnioperitoneal connection without the mediation of the umbilical cord. Exomphalos with abdominal wall defect and serve scoliosis were characteristic components of this anomaly, that provided important clues in differentiating other similar anomalies. Other associated anomalies included neural tube defect, intestinal atresia, genitourinary and skeletal defects, pulmonary hypoplasia, single umbilical artery and narrow-spaced chest and abdomen, etc. These findings strongly suggest that anomaly of body stalk represents mechanical teratogenesis due to early amnion repture and subsequent effect, and should be categorized into amniotic band disruption syndrome.
Pregnancy ; Female ; Humans ; Teratogens

To investigate the inflammatory cytokine production of human epithelial cell lines stimulated with uropathogenic E. coli strains showing 3 different adherence patterns, differential expression of inflammatory cytokine (IL-1a, IL-lB, IL-8, TNFa, and TGFB) mRNA were detected by RT-PCR. IL-1a, IL-1B, IL-8, and TGFB mRNAs constitutively expressed in epithelial cell lines, but not TNFa. The expression of IL-1a and IL-1B mRNA was increased in J-82 cells stimulated with E. coli strains showing DA, LA, or AggA pattern. The expression of IL-8 mRNA was increased, whereas TGFj3 mRNA was decreased in J-82 cells stimulated with E. coli strain showing AggA.pattern. Treatment with crude bacterial adhesins (CBA) isolated from E. coli strains showing DA or LA pattern increased IL-la, IL-lB, IL-S, and TGFj3 mRNA expressions in J-82 cells and HeLa cells. IL-la, IL-lB, and TGFB mRNA expressions were decreased in epitheUal cells stimulated with CBA from E. coli strain showing AggA pattern, whereas IL-8 mRNA expression was significantly increased. The expressions of cytokine mRNAs showed little differences between epithelial ceRs used, but great differences between CBA from DA or LA and AggA strain. LPS stimulation was little changed cytokine mRNA expressions in epithelial cells. This study suggests that cytokine gene expression of epithelial cells by the bacterial stimulation mainly depends on the bacterial adhesins recognized by the respective receptors of epithelial cells.
Adhesins, Bacterial ; Epithelial Cells* ; Gene Expression ; HeLa Cells ; Humans ; Interleukin-8 ; RNA, Messenger ; Uropathogenic Escherichia coli*

Foreign body reaction of tissues is frequently encountered in routine surgical pathology, whether they contain foreign body or not. Though their presence or identification may be the important clue for the legal problems as in malpractice, and the identification of foreign body will give us the chance for reconstruction of patients' medical history, pathologists often overlook or neglect what they are. Besides curiosity, the description of individual foreign body and common site for its occurence will be of great help. Therefore, we classify and describe the features of 182 foreign bodies collected in practice of surgical pathology during 1979~82.

No abstract available.

During the past two decades, silicone (polydimethylsiloxane) has become one of the most extensively applied biomaterials. Although pure silicone is relatively inert and usually causes only minimal tissue reactions, it has been reported to evoke a definite foreign body reaction. We studied five cases of silicone-induced granulomas in various sites; two in the breast, one in the breast and axillary lymph nodes, one in the subcutis of the abdomen, back and extremities and one in the eyeball, to illustrate the salient histopathologic features of reactions to silicone with particular emphasis to its differences from paraffin granuloma. For this, 17 paraffinomas were also studied. Tissue reaction to silicone liquid and gel was characterized by numerous round to oval empty cystic vacuoles, mild to moderate fat necrosis, foreign body reaction, a variable degree of mononuclear inflammatory cell infiltration and mild focal fibrosis. The cystic spaces were relatively uniform and showed a snow-man like appearance. In contrast to the silicone granulomas, the paraffinomas, also refered to as sclerosing lipogranulomas showed diffuse sclerosis and frequent calcification around the cystic vacuoles. The cystic spaces in paraffinomas were swiss cheese-like configuration, and the content of the cystic spaces was dirty and frequently calcified. However, there were certain similarities between these two types of granulomas particularly in the early phases of the reaction, therefore, the history of silicone injection or implant, is sometimes critical to the diagnosis of silicone granuloma. Despite great technologic advances in the manufacturing of prostheses and medical equipment, droplets and/or particles of silicone still escape into the body tissues in a variety of ways; therefores, the pathologist should always wonder whether the histologic reaction observed is due to silicone or to some other foreign material including paraffin.

BACKGROUND: Hydrops fetalis is defined as abnormal accumulation of serous fluid in two or more fetal compartments, and this malady is known to be associated with various pathologic conditions. METHODS: We collected 149 cases of hydrops fetalis out of 2,312 autopsies, and we tried to elaborate the underlying causes of hydrops fetalis. The diagnosis was based on the material from either antenatal termination or intrauterine death. RESULTS: The relative incidence of hydrops fetalis was 6.44% of all the pediatric autopsies we performed. The gestational age was evenly distributed from 18 to 33 weeks, except for 30 to 31 weeks. There was no sex difference in the incidence of hydrops fetalis. The main causes were cardiovascular diseases (30.9%), cystic hygroma (13.4%), chromosomal anomaly (8.05%), thoracic conditions (7.38%), followed by urinary tract malformation (4.03%), infection (4.03%) and anemia (3.36%). The most common chromosomal anomaly was Turner syndrome and the second one was Down syndrome. CONCLUSION: Since various conditions can be the cause of hydrops fetalis, pathologists should pay attention to elaborate the underlying causes in every single autopsy.
Anemia ; Autopsy* ; Cardiovascular Diseases ; Diagnosis ; Down Syndrome ; Edema* ; Fetus ; Gestational Age ; Hydrops Fetalis* ; Incidence ; Lymphangioma, Cystic ; Sex Characteristics ; Turner Syndrome ; Urinary Tract

Five fetal maxillas were obtained from the autopsy file of fetal postmortem examination, and were examined by serial micro-sections of frontal plane and horizontal plane. Especially the area around the incisive canal of the maxilla was carefully observed. The results are as follows. 1) In 5 fetal maxillas extra-dental laminas and supernumerary tooth germs which are severely malformed in shape are found in the dilated incisive canal, where prominent vessels and nerves are distributed. 2) The supernumerary tooth germs disclose almost normal histo-differentiation of odontoblast and ameloblast, and there shows relatively abundant perifollicular fibrosis in the place of perifollicular bone. 3) It is observed that the over-growth of the extradental lamina from the dental ridge of deciduous central incisor frequently tends to direct toward the incisive canal that includes prominent vessels and nerves.

The patient was a 7 months old Korean girl, who was admitted to Department of Ophthalmology of the Seoul National University Hospital, with 6 months history of the left eyelid swelling and exophthalmos. Visual acuity and ocular movement were unaffected. Orbit CT revealed 1.5x.5 cm sized well circumscribed intraconal mass in the left retrobulbar space with pressure erosion of adjacent bone. At surgery, the mass was pinkish gray and firm, and was adherent to adjacent tissue. The whole mass could not be removed, and a local excision was done.