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

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

No abstract available.
Kidney*

Beckwith-Wiedemann syndrome is a rare clinical entity characterized by exomphalos, macroglossia, macrosomia, and renal hyperplasia/dysplasia. Although its entity is established, its etiology and obligatory features have not been settled. We report an autopsy case with the unusual involvement of the salivary gland. This infant was born to a 37-year-old mother as a normal full-term spontaneous delivery. At 11 days of age she developed with purulent eye discharge and weak sucking, and died suddenly. At autopsy the baby weighed 2,630 gm and the head circumference was 35 cm. She showed thick and prominent skin folds, bilateral aural fissures, macroglossia, hepatomegaly, cardiomegaly, dysmorphic kidneys, and nesidioblastosis. Both kidneys showed dysplastic tubules and hyperplastic cortical tissue enclosing the medulla. In this case there were characteristic findings in major and minor salivary glands with both acinar and ductal hyperplasia, and hypertrophy of mammary glands. Besides, she had generalized depletion of subcutaneous fat, immature buccal fat, patent ductus arteriosus, hyperlobation of the right lung, two accessory spleens, and hyperplasia of basophils and chromophobes in the pituitary gland. The lungs showed diffuse interstitial pneumonia and multiple fibrin thrombi. There were no adrenal cytomegaly, umbilical hernia and exophthalmos.
Infant ; Male ; Female ; Humans

Since early 1980's high resolution ultrasound has been world-widely used for detection of cranial lesions ininfants but not widely used in korea. Authors prospectively analysed ultrasonographic findings of 107 cases whichwere confirmed by CT, autopsy or follow-up studies as supplement. the distribution of 107 cases was intracranialhematoma 40 cases, hydrocephalus 36 cases, hypoxic-ischemic encephalopathy 10 cases, porencephalic cyst 5 cases,cephalhematoma 5 cases, agenesis of corpus callosum 4 cases, medulloblastoma 2 cases and each one case of A-Vmalformation, intraventricular cyst, Dandy Walker cyst, lipoma and hydranecephaly. We could conclude thatneurosonography of infants was very useful and effective method in detection of cranial lesions such asintracranial hematoma, especially germinal matrix hemorrhage or intraventricular hemorrhage in preterm infant,hydrocephalus, hypoxic-ischemic encephalopathy and congenital anomalies.
Agenesis of Corpus Callosum ; Autopsy ; Follow-Up Studies ; Hematoma ; Hemorrhage ; Humans ; Hydrocephalus ; Hypoxia-Ischemia, Brain ; Infant ; Korea ; Lipoma ; Medulloblastoma ; Methods ; Prospective Studies ; Ultrasonography ; Walkers

A 45-Year-old para 1-0-1-1 had submucosal myoma diagnosed by ultrasonogram and hysteroscopic examination. During 60 minutes hysteroscopic myomectomy, 8,000 ml of sorbitol-mannitol distention medium was used and 6,500 ml collected, resulting in a deficit of 1,500 ml. The patient received Ringer`s lactate 1,000 ml intravenously. Her urine output was 400 ml. Blood loss was 40 ml. After operation, hyponatremia(115 mEq/L) was developed and pulmonary edema was also diagnosed clinically at that time and later confirmed by chest radiograph. The judicious administration of diuretics and normal saline fluid replacement to prevent overcorrection to hypernatremia resulted in return of serum electrolytes to normal level and resolution of the pulmonary edema by postoperative day 1. We have experienced a case of pulmonary edema during hysteroscopic myomectomy with sorbitol-mannitol distention medium, which is presented with a brief review of literatures.
Diuretics ; Electrolytes ; Humans ; Hypernatremia ; Hyponatremia ; Lactic Acid ; Middle Aged ; Myoma ; Pulmonary Edema* ; Radiography, Thoracic ; Ultrasonography

To study the basic pathological changes of small intestine in metagonimiasis, light- and electron microscopic studies were made, using a total of 21 cats which were experimentally infected with metacercariae of Metagonimus yokogawai. The metacercariae were obtained from naturally infected sweetfish (Plecoglossus altivelis) by digestion technique. The cats were divided in control, light-infection(10,000 metacercariae infected) and heavy-infection(50,000 metacercariae infected) groups. Cats were killed at the 5th, 10th, 15th day, and 4th, 8th and 10th week after the infection. And the small intestine was prepared for the study. Pathological studies comprised gross examination, worm distribution pattern, light microscopic examination and both transmission and scanning electron microscopic examinations. The results obtained were summarized as follows. Gross morphologic changes were the most marked during the first 2 weeks after infection. The gross abnormalities were severer in the heavily infected animals. The changes were dryness and listlessness of serosal surface due to dehydration, mushy and/or watery intestinal content, effacement of transverse nodes and enlargement of mesenteric lymph folds and Peyer's patches. After 4 weeks of infection, these changes became less marked showing a tendency to return to normal. The sectioned flukes were distributed from duodenum to proximal ileum. However, individual variation was marked in distribution. In the heavy-infection group, the locality of parasitism tended to extend more distally. The locality of M. yokogawai in the intervillous space was mostly in the lower-most portion of intervillous space, where they compressed and eroded epithelial cells probably due to mechanical damage to the structure. Very rarely the worms were found in lumen of Lierberkuehn's crypt, and reaching, in two occasions, into proprial lymphoid tissue. Light-microscopically the lesion was restricted in mucosa: Early mucosal changes were shortening, blunting, fusion, and thickening of the villi, crypt hypertrophy with consequent decrease of villus/crypt ratio, as well as stromal changes of edema, capilliary ectasia and marked inflammatory cell infiltration of lymphocytes and plasma cells. Goblet cells were markedly reduced in number as with depletion of its cytoplasmic content. In the later stages of infection, mucosa restored its normal configuration in spite of persistent parasitism of the worms. At the infection stage of 5-15 days, there was significant shortening of the microvillous height with varible destruction of glycocalyx in electron microscopic examination. With lapse of infection time, microvilli became to restore the normal pattern. With these morphological changes, it appears that diarrhea in experimental metagonimiasis would be related to the decrease of absorptive surface of the small intestine particularly in the early phase of infection. The significant changes seen in villi and microvilli might be due to massive intrusion or invasion of Metagonimus worms into the crypts, causing direct mechanical and possible host-immune response to the small bowel mucosa.
parasitology-helminth-trematoda ; metagonimiasis ; Metagonimus yokogawai ; pathology ; cat-intestine ; edema ; lymphocytes ; plasma cells ; goblet cell

Optic nerve gliomas manifest either as a solitary form or a component of von Recklinghausen's neurofibromatosis. The reported incidence of coexistence with neurofibromatosis varies from 12% to 70%. Usually there are no significant cytological differences between the gliomas that accomapny the disease and those that are deemed to be solitary manifestations. The only possible difference between them is the apparently more common association, with the former, of extensive arachnoid hyperplasia and of a more florid local gliomatous infiltration into the leptomeninges, altogether resulting in perineural thickening. Our cases were 8 and 6 years old girls, respectively, presented with slowly progressive proptosis for 4 years and visual disturbance for 2.5 months. There were multiple cafe au lait spots on their trunks, and case 2 showed Lisch nodules in the iris. MRI of brain revealed unilateral optic nerve thickening with involvement of chiasm or multiple intracranial lesions. Resection of optic nerve tumor was performed. Microscopically, variable degree of tumorous change was seen. Most typically enlarged area was composed of intraneural and perineural portions surrounded by a layer of intact dura. Intraneural tumor revealed proliferation of elongated, spindle-shaped pilocytic astrocytes in intertrabecular spaces and distention of the pial septa with fibrovascular thickening. Another segment had areas with reactive gliosis. Perineural tissue was considerably thickened and, associated with proliferation of meningothelial cells and fibroblasts intermingled with astrocytes and Rosenthal fibers. There were increase of the optic nerve diameter and distention of the overlying dura. Foci of arachnoid cell hyperplasia were noted in both cases, although differed in degree. Immunohistochemically, the tumor cells expressed glial fibrillary acidic protein in intraneural and perineural portions particularly in case 2.
Incidence

In evaluating the early state of shock patients and their response to treatment, generally vital signs or additional hemodynamic values were used. Vital signs are easily obtained and repetitious values or continous monitoring is possible, whereas it cannot evaluate the patient's status properly nor is it a good prognostic factor. Meanwhile, additional hemodynamic values are obtained from pulmonary artery catheterization. But this procedure is difficult to proceed in the emergency room. Since central venous oxygen saturation has the advantage of being easily obtained and acts as a good prognostic factor for shock patients, we intended to prove its efficacy. From 1997 May to October, 50 patients admitted to Yonsei University Young Dong Severance Hospital in shock state, with systolic blood pressure below 90mmHg, were evaluated. Central venous catheter was inserted to obtain central venous oxygen saturation, serum lactate, base excess value, and its' initial value compared with the prognosis. The central venous oxygen saturation and base excess were low, while serum lactate value was high in the nonsurvival group. And in ROC(relative operating characteristic) curve, the AUC(area under curve) of central venous oxygen saturation was larger than the others. In comparing the MOF(multi-organ failure) group with the non-MOF group, the MOF group had a lower central venous oxygen saturation and base excess, and a higher serum lactate level, whereas in ROC curve, the AUC of base excess was larger than the others. Therefore, in estimating the prognosis of shock patients, the early central venous oxygen saturation proved to be a good prognostic factor.
Area Under Curve ; Blood Pressure ; Catheterization, Swan-Ganz ; Central Venous Catheters ; Emergency Service, Hospital ; Hemodynamics ; Humans ; Lactic Acid ; Oxygen* ; Prognosis ; ROC Curve ; Shock* ; Vital Signs

No abstract available.
Alexander Disease*