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

No abstract available.
Anal Canal*

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

No abstract available.
Hysteroscopy*

No abstract available.
Hysteroscopy*

In order to evaluate the efficiency of serological typing of A. baumannii in practical application, a total of 63 strains of A. baumannii and 234 strains of Gram-negative, lactose non-fermenting bacteria were tested with polyclonal rabbit immunized sera (RIS) against heat-killed A. baumannii strains by slide agglutination tests. Six typing sera of RIS were finally obtained after the checkerboard agglutination test and reciprocal cross-absorption. Species identification of sixty-three strains of A. baumannii were confirmed by ribotyping. Forty-seven (74.6%) of the 63 strains of A. baumannii showed strong positive reaction by slide agglutination tests. Thirty-nine strains could be serotypable and thus classified into 6 distinct serovars of A. baumannii, but 8 strains were unable to classify into specific serovar. Serovar 4 was the most frequent arbitrary serovar and included 17 strains among the 39. When slide agglutination tests were performed with 50-fold diluted pooled polyclonal RIS, there was no cross-reactions except one of E. coli strain among 234 strains of various Gram-negative lactose non-fermenting. Although each profile of LPS-gel electrophoresis of A. baumannii appeared to be unrelated with serovar, the patterns of western-blot of LPS after immunostaining with homologous RIS showed serovar-specificity. Several fractions of low molecular weight LPS showed cross-reaction with antisera of other serovars. In conclusion, the sensitivity and specificity of serological identification of A. baumannii strains were 74.6% and 99.6%, respectively. This result suggests that serotyping is a useful method for the identification of A. baumannii strains as well as is the epidemiological tool to trace back the source of the nosocomial outbreaks.
Acinetobacter baumannii* ; Acinetobacter* ; Agglutination Tests ; Bacteria ; Disease Outbreaks ; Electrophoresis ; Immune Sera ; Lactose ; Molecular Weight ; Ribotyping ; Sensitivity and Specificity ; Serotyping

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

This study investigated the efficacy and safety of melphalan, cyclophosphamide, and dexamethasone (MCD) as a salvage regimen for heavily treated relapsed or refractory multiple myeloma patients. We retrospectively analyzed a total of 27 patients who received the MCD regimen between April 2011 and November 2013. The MCD regimen consisted of oral melphalan 6.75 mg/m² on days 1–4, once-weekly dose of oral cyclophosphamide 300 mg/m2 and dexamethasone 20 mg/m² on days 1–4 and days 15–18. Each cycle was repeated every 28 days. The median age of the patients was 66 years and the MCD regimen was initiated at a median 37.7 months from diagnosis. Patients received a median of five regimens including autologous stem cell transplantation. The overall response rate was 25.9% (very good partial response 3.7%, partial response 22.2%) and 8 (29.6%) patients achieved a minor response. Median progression-free survival was 5.6 months (95% confidence interval [CI], 4.2–8.5) ; overall survival 11.7 months (95% CI, 5.4–16.6). Grade 3 or 4 neutropenia and thrombocytopenia were observed in 51.8% and 33.3%, respectively. Although the overall response rate is relatively low, the MCD regimen may have a role as a bridge to a novel regimen in heavily pretreated patients with MM.
Cyclophosphamide* ; Dexamethasone* ; Diagnosis ; Disease-Free Survival ; Humans ; Melphalan* ; Multiple Myeloma* ; Neutropenia ; Retrospective Studies ; Salvage Therapy* ; Stem Cell Transplantation ; Thrombocytopenia

No abstract available.
Pregnancy*

OBJECTIVES: There have been several studies documenting the changing level of the conus throughout infancy and childhood, but there is only a little detailed study that documents the range of conus positions in a living adult population, especially in Korean, without spinal deformity. METHODS: we made a sequential study of magnetic resonance images of the lumbar spine to determine the variation in position of the conus medullaris in 650 living korean adults population without spinal deformity who checked MRI to identify the cause of low back pain. The study population consisted of patients over the age of 16 years. A T1-weighted, midline, sagittal image was reviewed for identifying the postion of conus. This location was recorded in relation to the upper, middle, or lower third of the adjacent vertebral body or the adjacent intervertebral disc. RESULTS: The study group consisted of 305 men(47%) and 345 women(53%) with a mean age 45.9 years(range, 16-79 years). The conus existed commonly at the middle third of L1(131cases, 20.2%), at the L1-2 intervertebral space(129cases, 19.8%), and the lower third of L1(123cases, 18.9%). The mean position of conus was the lower third of L1(range, middle third of T12 to middle third of L3). Conclusions:The mean position of conus was at the lower third of L1(range, middle third of T12 to middle third of L3). This results was same as that of foreign study. Our results of living korean adult population could allow for safe clinical procedures such as lumbar puncture, spinal anesthesia, and help to explain the differences among observed neurologic injuries from fracture-dislocation at the thoracolumbar junction.
Adult* ; Anesthesia, Spinal ; Congenital Abnormalities ; Conus Snail* ; Humans ; Intervertebral Disc ; Low Back Pain ; Magnetic Resonance Imaging* ; Spinal Puncture ; Spine