Incontinentia pigmenti achromians (Ito) begins during childhood and is characterized by progressive appearance of bizarre patchy or whorl-like hypopigmentation without any preceeding or associated inflammatory changes in a fashion that resembles a negative piture of pigmentation in patients with incontinentia pigmenti. Mental, bony and ocular symptoms are not infrequently found in association with incontinentia pigmenti achromians. The incidence is high in the female sex, and there is no hereditary background. A 24-year-old Korean soldier was first seen in May 1974 in dermatologic clinic of Capital Armed Forces General Hospital. Bizarre, reticulated, linear and whorllike hypopigmented macular lesions were noted. On the skin of the trunk and both extremities. The lesions started at the age of 4 year and developed without any inflarnmatory signs Family history did not disclose any type of pigmentation The disorder, and mental, bony and occular manifestations were not found. Biopsy specimen demonstrated that the amount of melanin in the basal layer was decreased in the hypopigmented area, and reveals neither inflammatory changes nor dropping off of melanin granules into the dermis.
Arm ; Biopsy ; Dermis ; Extremities ; Female ; Hospitals, General ; Humans ; Hypopigmentation ; Incidence ; Incontinentia Pigmenti ; Melanins ; Military Personnel ; Pigmentation ; Pigmentation Disorders ; Skin ; Young Adult

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

Experimental allergic encephalomyelitis (EAE) has been a well established animal model of postvaccinatal demyelinating diseases occurring in humans. Therefore elucidation of its pathogenesis would be very critical for the understanding of various human demyelinating diseases including multiple sclerosis. This study was performed to characterize the infiltrating cells in inflammatory sites and analyze the nature of the damage of blood brain barrier in experimental allergic encephalomyelitis. Experimental allergic encephalomyelitis was produced by administering homologous spinal cord homogenate together with complete Freund's adjuvant in guinea pigs. Immunostainings on guinea pig IgG, IgM, IgA and muramidase were performed by peroxidase-antiperoxidase or indirect immunofluorescent methods. The blood-brain barrier change was assessed by administering fluorescent Evans blue. Following results were made. In juvenile animals, both clinical findings and histopathologic changes were first noted by 3 weeks after injection and progressed during the whole experimental period. However, these findings were delayed in onset and low in incidence in adult animals. The clinical and pathologic changes started from the caudal portions and extended rostrally. The blood-brain barrier (BBB) was damaged and progressed starting also from the caudal portion of the spinal cord. The BBB changes were more severe in young animal than adult animals. Those changes preceded th histologic alterations. It is suggested that the BBB susceptibility is responsible for the caudal onset of histologic changes. Although the lesion has been thought to be induced by T-cell mediated hypersensitivity, infiltrating cells consisted mainly of muramidase positive histiocytes. A few immunoglobulin positive B cells or plasma cells could also be demonstrated in the lesion. The former usually infiltrated the parenchyme and the latter remained around the small or medium-sized vessels.
Adult ; Male ; Female ; Humans ; Animals ; Incidence

Retropsoas space below the level of kidney has been suggested as a portion of inferior extensions of perirenal and anterior and posterior pararenal spaces. With this being true, the space may play an important role in disease extension. A study was performed to verify the existence of retropsoas peritoneal recess by means of identifying the extension of bowel loops into this space. Abdominal CT scans of 146 cases evaluated retrospectively revealed extension of 5 small bowel and 7 large bowel loops (6 descending and 1 ascending colons)(n=12/8.2%) into the retropsoas space verifying its existence. Since pathologic collection within the retropsoas space might be falsely inter preted as a retroperitoneal pathology and percutaneous uroradiologic intervention could result in intraperitoneal injury or contamination without the knowledge on the existence of this space, observation of this space is essential in CT scans.
Kidney ; Pathology ; Retrospective Studies ; Tomography, X-Ray Computed

Cystadenomas are benign neoplasm in which the epithelium demonstrates adenomatous proliferation. Several morphologic variants of cystadenomas have bean described, including papillary-oncocytic, mucous, and seromucous subtypes. Cystadenomas arising from salivary glands are very rare and seromucous cystadenoma of parotid gland has not been reported in Korea so far. Cystadenomas of the parotid gland usually Present as asymptomatic slowly growing mass and those of the minor salivary glands produce smooth nodules that may be compressible. We performed a superficial parotidectomy in a patient with a seromucous cystadenoma. This may be the first report of seromucous cystadenoma of carotid gland in Korea.
Cystadenoma* ; Epithelium ; Humans ; Korea ; Parotid Gland* ; Salivary Glands ; Salivary Glands, Minor

PURPOSE: It has been known that there are several areas of T2 hyperintensities in normal white matter of brain, such as terminal zones of myelination, ependymitis granularis, ones of posterior internal capsule, and perivascular space. The aim of our study is to demonstrate another region of T2 hyperintensities in normal pediatric age group. MATERIALS AND METHODS: We have studied brain MR for 10 normal volunteers and 35 patients without having intracranial lesions in pediatric age group(3-19 years). RESULTS: In 5 among 45 cases, focal T2 hyperintensities were seen in the parietal periventricular white matter beneath the postcentral gyri. They were noted as poorly defined, 5--10mm sized areas of increased signal intensities on T2 weighted axial images. They were also characterized by bilateral, posteromedially oriented, short band-like or oval areas. Interestingly, they were directly continuous with the T2 hyperintensity of posterior internal capsule. In spite of the relatively high frequency in the pediatric population as in our study, this finding has not been reported in the asymptomatic adults. CONCLUSION: The results show that the bilateral anterior parietal hyperintense areas may be another terminal zones of delayed myelination affecting the parietopontine tract. They should be differentiated from pathologic T2 hyperintensities by their characteristic findings.
Adult ; Brain ; Healthy Volunteers ; Humans ; Internal Capsule ; Myelin Sheath ; Rabeprazole*

No abstract available.
Thioctic Acid*

Epithelial-myoepithelial carcinoma of intercalated duct(origin) is a recently described tumor characterized by its typical biphasic pattern of central duct like cell and peripheral clear cell. We described a case of epithelial-myoepithelial carcinoma in a 10-year-old boy. Microscopically, the tumor showed typical biphasic pattern, diffuse proliferation of clear cells and linining epithelial cells of tubular structures. Immunohistochemically, the clear cell showed positive reaction to S-100 protein, and the epithelial cells expressed cytokeratin indicating myoepithelial and epithelial differentiation respectively. Biphasic differentiation of the tumor cells could be also proved by electronmicroscopic study.

Biofeedback is the treatment of choice for functional defecation disorders such as idiopathic chronic constipation and neurogenic fecal incontinence. The pre-existing biofeedback systems have many disadvantages. The aims of current project are, first, to develop the biofeedback system into the application software in the Windows environment, and, second, to assess the possibility of clinical usage for patients with functional defecation disorders. The hardware and software of the BASCO (Biofeedback Anal Sphincter Control) system were based on the signal measurement and signal processing of anal sphincter EMG (Electromyography). BASCO system was applied to 5 normal healthy controls and 20 patients with functional defecation disorders. Patients group was categorized as constipation group (N1=15) and incontinence group (N2=5). With use of current system, EMG-based biofeedback therapy was performed, and the outcome was analysed. Anal EMG signal data was processed by the software, and displayed in the monitor of personal computer. The software of EMG-display and database management were adequately operated. In N1 group, a paradoxical elevation or equalized activity of anal EMG pattern was shown in the simulated defecation. In N2 group, low electrical activity was shown. These findings were used for the EMG-based biofeedback therapy as a pilot study. The clinical symptoms were improved in 12 of N1 group and 3 of N2 group in the period of 3.7 (range, 1~12) months follow-up. In Conclusion, newly-developed BASCO system was adequately operated in the volunteer and patients groups. The multi-tasking and multi-processing functions were adequately shown in the real time. Current results could be used for clinical appraisal. Specifically, this system could be used for the practical application of biofeedback therapy in the patients with chronic constipation or fecal incontinence.
Anal Canal* ; Biofeedback, Psychology* ; Constipation ; Defecation* ; Fecal Incontinence ; Follow-Up Studies ; Humans ; Microcomputers ; Pilot Projects ; Volunteers

No abstract available.
Helicobacter pylori* ; Helicobacter* ; Urease*