No abstract available.
Classification* ; Myelodysplastic Syndromes*

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*

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

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.

Waardenburg syndrome is a very rare auditory-pigment syndrome with autosomal dominant inheritance. In his first report in 1951, Waardenburg characterized this syndrome as dystopia canthorum, a high broad nasal bridge, synophrys[confluent eyebrows], heterochromia iridid, a white forelock or early graying, and sensory neural deafness. We observed a case of Type 2 Waardenburg syndrome with openangle glaucoma and, therefore report with literature review.
Deafness ; Glaucoma ; Glaucoma, Open-Angle* ; Waardenburg Syndrome* ; Wills

A three year old girl with a left renal teratoid Wilms tumor is reported. The tumor was located both inside and outside the kidney parenchyme, to form a well encapsulated mass containing two lobulated solid and cystic masses. The tumor consists predominantly of otherwise typical Wilms tumor irregularly mixed with teratoid tissue elements such as intestinal tract, mucous glands with argentaffin cells, goblet cells and transitional epithelium. These heterologous elements were regarded as diverse epithelial differentiation of totipotent cells in certain nephrogenetic period, and this tumor was considered to be hest called "teratoid Wilms tumor".

We report an autopsy of a male fetus that showed multiple congenital anomalies that could best be designated as Meckel-Gruber syndrome. The fetus was born dead at the gestational age of 38 weeks. His parents denied any history of congenital malformation. And the parity of the mother was 0-0-0-0, but she had the past history of receiving herb medication for common cold. The congenital anomalies found in this case consited of occipital meningoencephalocele, midline cleft palate, bifid epiglottis, hepatic fibrosis, choledochal cyst, bilateral polycystic kidneys, postaxial polydactyly of both hands and feet, aplasia of the left testis, secundum type atrial septal defect and patent ductus arterious. This malformation syndrome is rare and lethal. The prenatal diagnosis should be made by ultrasound study or analysis of the amniotic fluid for alpha-feto protein during intrauterine period. The kidneys showed Potter type III cystic change and there was a characteristic hepatic fibrosis.
Male ; Humans