A human embryo obtained incidentally from a hysterectomy specimen of a 36 year old woman who was operated for chronic pelvic inflammatory disease, was serially sectioned and reconstructed with 104 section slides. This embryo was characterized by a thick corneal body with well developed Descemet's endothelium, the optic nerve having vascular canal, the cochlear turning a little more than one time, the hypophysis with threadlike stalk, the vomeronasal organ having intermediate long narrow canal, the submandibular gland with deep lumen, the kidney with short secretory tubules and few large glomeruli and a well formed osseous band in the cartilage and bone. From above findings, it was concluded that this embryo belongs to the age group XXIII of Streeter's developmental horizon.
Female ; Humans

Scanning electron microscopic(SEM) examination of hair is an efficient and definite method for the diagnosis of hair anomaly. The Hair specimen is placed on a stub, gold coated, and directly viewed without prior fixation or treatment for identification. We have analyzed 25 cases of scalp hair and 2 cases of eyebrow that were sampled with the suspicion of hair anomalies at Seoul National University Hospital during the last 7 year period from January 1988 to June 1992. All 27 cases had certain abnormalities of the hair. They consisted of monilethrix(1 case), hair casts(4 cases), pili torti(1 case), uncombable hair syndrome(1 case), trichorrhexis nodosa(5 cases), woolly hair(5 cases) and other anomaly(1 case). Some cases of them were mixed with two or three anomalies in hairs. Their combinations were; hair casts and woolly hair(1 case), hair casts and pili torti and uncombabla hair syndrome(1 case), hair casts and uncombable hair syndrome and trichorrhexis nodosa(1 case), trichorrhexis nodosa and uncombable hair syndrome(3 cases). Most of these defects included brittleness of scales and distortion of the hair shaft.

PURPOSE: The purpose of this study was to describe the clinical outcome after pancreatcetmy and its relationship with pathological appearances and clinical features in patients with persistent hyperinsulinemic hypoglycemia of infancy(PHHI). METHODS: Medical records of 10 patients(9 males and 1 female, mean age:40.4+/-1.5 months) who were diagnosed as PHHI and underwent pancreatectomy from 1988 to 2000 were reviewed. Clincal and biochemical data were recorded. Subjects were classified arbitrarily into early-onset or late-onset group according to age of onset. Pathologic appearance of pancreas was divided into 2 forms:diffuse or focal. The former had a focal pancreatic adenomatous hyperplasia and the latter was characterized by increased number of betacells with similar distribution seen in normal neonates. RESULTS: One patient had focal, and nine had diffuse lesions. After near-total pancreatectomy, 4 patients(40.0%) showed complete response, 4(40.0%) had persistent hypoglycemia, and 2(20.0%) developed diabetes mellitus. As neurological sequelae, 6 patients(60.0%) had persistent seizures, and 6(60.0%) had delayed motor and speech development. No clinical or biochemical factors related to postoperative outcome were found. CONCLUSION: This data indicate that early diagnosis of patients who present with hypoglycemic symptoms in infancy, especially early in life, and development of more effective therapy are warranted, because there is no clinical or biochemical factor predicting final outcome after near-total pancreatectomy and only 40% of patients with PHHI remained euglycemic after surgery with possible severe neurological sequelae.
Age of Onset ; Congenital Hyperinsulinism* ; Diabetes Mellitus ; Early Diagnosis ; Female ; Humans ; Hyperplasia ; Hypoglycemia ; Infant, Newborn ; Male ; Medical Records ; Pancreas ; Pancreatectomy* ; Seizures

PURPOSE: Mutations in the p53 gene have been recognized in brain tumor, and clonal expansion of p53 mutant cells has been shown to be associated with glioma progression. However, studies on the p53 gene have been limited by the need for fresh frozen tissues. We have tried a method utilizing polymerase chain reaction (PCR) for the direct DNA sequecing of the p53 gene using a single 10 m paraffin-embedded tissue section. We applied this method to detect for p53 gene mutations in exons 5~8 in human astrocytoma utilizing paraffin-embedded tissues. MATERIALS AND METHODS: Twenty paraffin blocks containing tumor were selected from surgical specimens from twenty different cases. Tumors included 10 astrocytomas and 10 anaplastic astrocytomas. Ten controls were also selected among autopsy cases showing normal brain in light microscopy. The tissue section on the stained glass slide was used to guide microdissection of an unstained adjacent tissue section to ensure above 90% of the tumor cell population for p53 mutational analysis. RESULT: Mutation in the p53 gene was identified in 1 of 10 (10%) anaplastic astrocytomas. Mutations in the p53 gene were identified in 1 of 10 cases (10%) by PCR and direct DNA sequencing. Mutation in exon 7 resulting in amino acid substitution was found in one anaplastic astrocytoma (codon 245, GGC-->GAC: glycine-->aspartic acid). Ten control cases, ten astrocytomas and nine anaplastic astrocytomas were confirmed to be negative by direct sequencing of amplified DNA. CONCLUSION: This study demonstrates the feasibility of evaluating p53 gene mutations in archived astrocytoma specimens using PCR and direct DNA sequencing on paraffin sections. Application of this method should facilitate investigation of the role of p53 gene mutations in tumor biology.
Amino Acid Substitution ; Astrocytoma ; Autopsy ; Biology ; Brain ; Brain Neoplasms ; DNA* ; Exons ; Genes, p53* ; Glass ; Glioma ; Humans ; Microdissection ; Microscopy ; Paraffin ; Polymerase Chain Reaction ; Sequence Analysis, DNA*

This study is based on 155 patients of congenital me colon. For the diagnosis, 93 cases were histologically proven and the remaining 63 cases were diagnosed on clinical basis including barium enema or surgical gross findings. On histologic examination, 80 cases(86%) showed typical features of absence of ganglion cell in the myenteric plexus and the 13 cases(14%) had atypical features which were segmental absence Of ganglion cell in one case. There we 127 males(82%) and 28 females(18%). The age at diagnosis was younger than 30 days in 87 cases(56%), I month to 1 year in 39 cases(25%) and older than I year in 29 cases(18%). The levels of aganglionosis were variable: short segment (rectosigmoid) in 134 cases(86%), intermediate segment (more proximal colon) in 14 cases(100%). and 6 cases(4%) had total aganglionosis. Common clinical presentations were abdominal distention, delayed meconium passage or bilious vomiting in neonate, and chronic constipation in infancy or childhood. Following initial colostomy or ileostomy, a definitive procedure was performed in 151 cases(Duhamel type in 150 cases; Soave type in 2 cases; Swenson type in 3 cases). Frequently associated problems after definitive procedure were persistent constipation(ll%) due to septum formation, fecaloma, remnant aganglionic segment and rectal stenosis. Overall mortality rate was 4%, and increased mortality was associated with enterocolitis(14%) which was the most frequent cause of death. The follow-up study longer than 3 months was available in 138 patients who underwent a definitive procedure(mean 2 year 11 months). Seventy-three cases(53%) had normal bowel function, 38cases(27.5%) had occasionally used enema or stool softners, and 27 cases(19.5%) had severe constipation or soiling. The bowel habit improved with time, and were considered normal in 60% of patients after follow-up more than 3 years. The results of definitive procedures for congenital megacolon including Duhamel operation was satisfactory, and long-term follow-up appeared an important and critical component of patients'care.
Child ; Male ; Female ; Infant, Newborn ; Humans ; Mortality ; Follow-Up Studies

PURPOSE: Periocular carbon dioxide ultrapulsed laser surgery can induce ocular damage. Herein we report a rare case of corneal burn after carbon dioxide ultrapulsed laser for nevus removal. CASE SUMMARY: A 25-year-old female patient presented with ocular pain, decreased visual acuity, and metamorphopsia in the left eye after laser surgery for nevus removal at a dermatology clinic 4 days prior. The patient's best corrected visual acuity in the left eye was 20/100. Central corneal haziness and corneal epithelial defect were observed on biomicroscopic examination. Antibiotics, steroid and artificial tear eye drops were administered for treatment. Glasses were prescribed to correct refraction errors. After 2 months of treatment, the best corrected visual acuity in the left eye recovered to 20/50, and corneal epithelial defect was mostly healed, however corneal haziness remained. CONCLUSIONS: Carbon dioxide ultrapulsed laser is commonly used in dermatological treatments. The use of an eye shield is important during this procedure, and possibility of ocular surface damage from laser treatment should be considered.
Adult ; Anti-Bacterial Agents ; Burns* ; Carbon Dioxide* ; Dermatology ; Eyeglasses ; Female ; Glass ; Humans ; Laser Therapy ; Lasers, Gas ; Nevus* ; Ophthalmic Solutions ; Tears ; Vision Disorders ; Visual Acuity

A 48 year-old male with back pain and foot drop had been treated. Studies for myelomatosis were all negative. A plain lumbar spin X-ray showed an erosion of the left pedicle of the first lumbar vertebra. A myelography via lumbar and cisternal routes revealed total block with feather appearance at the upper level of the L2 and the lower level of the T12 vertebra. Pathologic evaluation confirmed the diagnosis of solitary myeloma of the bone. After undergoing almost total excision, followed by radiation, the patient was still doing well.
Animals ; Back Pain ; Diagnosis ; Feathers ; Foot ; Humans ; Male ; Middle Aged ; Multiple Myeloma ; Myelography ; Spine*

PURPOSE : To evaluate the MR imaging findings of bowel wall thickening in patients with minimal to moderate cirrhosis, and analyze their clinical significances comparing with laboratory findings. MATERIALS AND METHODS : We assessed retrospectively the MRI findings of 123 patients with minimal to moderate cirrhosis, and compared these with the clinical laboratory findings. We evaluated the involved sites and MR image findings of thickened bowel wall, as well as the presence of collateral vessels, ascites, and splenic size. These were compared with serum albumin and bilirubin levels, and prothrombin time. RESULTS : Gastrointestinal wall thickening was detected at 37 sites in 25 patients (20%), and more frequently detected in moderate cirrhosis (29%) than in minimal cirrhosis (17%). Jejunum and ascending colon were the most common sites of bowel wall thickening; each was involved at 22 and 9 sites, respectively. Ascending colonic wall thickening was more commonly detected in moderate cirrhosis than in minimal cirrhosis. The thickened bowel wall showed symmetric contour, high signal intensity on T2-weighted images, mixed iso- and low signal intensity on T1-weighted images, and homogeneous or target-like enhancement. Serum albumin level was significantly lower in patients with bowel wall thickening (3.3+/-0.9 g/dl vs. 3.9+/-0.7 g/dl;p=0.0024). Serum bilirubin level was significantly higher in patients with bowel wall thickening (1.7+/-1.0 mg/dl vs. 1.4+/-1.2 mg/dl; p=0.0160). Bowel wall thickening did not significantly correlate with the presence of collateral vessels, ascites, splenic size, and prolongation of prothrombin time. CONCLUSION : In minimal to moderate cirrhosis, the MR imaging evaluation of bowel wall thickening was useful for estimating the severity of cirrhosis and laboratory findings.
Abdomen ; Ascites ; Bilirubin ; Colon, Ascending ; Fibrosis* ; Gastrointestinal Tract ; Humans ; Jejunum ; Liver ; Magnetic Resonance Imaging ; Prothrombin Time ; Retrospective Studies ; Serum Albumin

A case of Nelson's syndrome in 23 year-old female, developing 6 years after bilateral adrenalectomy for Cushing's syndrome, is reported. Presenting symptoms were headache and hyperpigmentation of the skin and mucous membrane. Serum ACTH level was markedly elebated above 1000 pg/ml, but serum cortisol level diminished markedly. Transsphenoidal approach (TSA) with removal of gushing out hematoma and tumor underwent. Postoperatively, headache subsided and hyperpigmentation of the skin markedly improved.
Adrenalectomy ; Adrenocorticotropic Hormone ; Cushing Syndrome ; Female ; Headache ; Hematoma ; Humans ; Hydrocortisone ; Hyperpigmentation ; Mucous Membrane ; Skin ; Young Adult

A case of histiocytosis X with the involvement of pituitary stalk in a 5 year old boy was described. He presented with diabetes insipidus and the endocrinological study showed growth hormone deficiency. On computerized tomography and magnetic resonance imaging, the pituitary stalk was abnormally thick. Overlooking of the skull lesion on plain X-ray film led to an erroneous diagnosis of germ cell tumor'. Gross total removal of the lesion and chemotherapy were performed. In the differential diagnosis of suprasellar masses, this disease entity should be included. The importance of histological diagnosis of the infundibular lesion in cases of central diabetes insipidus is also emphasized.
Child, Preschool ; Diabetes Insipidus ; Diabetes Insipidus, Neurogenic ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Germ Cells ; Growth Hormone ; Histiocytosis* ; Histiocytosis, Langerhans-Cell* ; Humans ; Magnetic Resonance Imaging ; Male ; Pituitary Gland* ; Skull ; X-Ray Film