Miescher syndrome comprises congenital acanthosis nigricans, hypertrichosis, failure to thrive and short stature, dysmorphism especially of the jaws and oral cavity, insulin-resistant diabetes mellitus, and a characteristic general appearance. This report concerns a rare case of 12-year-old girl having insulin resistant diabetic mellitus with Miescher syndrome. The relevant literature was reviewed.
Acanthosis Nigricans ; Child ; Diabetes Mellitus* ; Failure to Thrive ; Female ; Humans ; Hypertrichosis ; Insulin ; Jaw ; Mouth

We report a 19-year-old man with hyperkeratosis of nipple and areola which is a part of an epidermal nevus. He did not have a history of endocrinopathy or synthetic steroid therapy. The lesion, a fingertip sized verrucous dark browncolored macule on left upper back, had spread into left-anterior chest wall and had involved nipple and areola to make them hyperkerstotic and verrueous. Histologic section from the on left areola showed hyperkeratosis, papillomatosis, acanthosis and increased pigmentation in the basal cell layer. Chernical peeling with 30% trichloroacetic showed favorable response.
Humans ; Nevus ; Nipples ; Papilloma ; Pigmentation ; Thoracic Wall ; Trichloroacetic Acid ; Young Adult

No abstract available.
Myxoma*

BACKGROUNDS: The Rh antigens are important m clinical practice. The classification of Rh phenotype is usually based on the antigen detection done by conventional serologic method, but it has many limitation such as delicate grading of antigen expression. Recently, Flowcytometry has been introduced in immunohematology to detect and quantitate cell bound immunoglobulins to assess blood cell antigens and related antibodies. So, we tried these method to detect Rh(D) antigen and measure its density and evaluated the possibility of clinical usage. MATERIALS AND METHODS: We performed a flowcytometric analysis for the expression of D antigen in D-positive, negative and weak D group in indirect immune fluorescence assay by using polyclonal antibodies. We measured the intensity of immunofluorescence as a degree of antigen density and analysed the difference of mean channel fluorescence value(MCF) among these groups. RESULTS: Weak D groups had the lower fluorescence than D-positive group, while D-negative sample showed the same fluorecence as negative ntrol. The difference of fluorescence intensity of D antigen between that of C antigen were not observed and were statistically insignificant. CONCLUSION: Flowcytometry appear to be a good tool for antigen density measurement in immunohematologic areas and shows the possibility of application to other aspect.
Antibodies ; Blood Cells ; Classification ; Fluorescence ; Fluorescent Antibody Technique ; Immunoglobulins ; Phenotype

BACKGROUDNS: In Korea, CPDA-1 solution is currently used for blood preservation and by this anticoagulant solution, RBCs can be preserved for up to 35 days at the refrigerate temperature. RBC additive solutions (AS-1, AS-3, AS-5, SAG-M, SAG-PM) are widely used in the other countries for longer preservation of RBCs (42 days). We studied the survival of transfused RBCs preserved in SAG-M additive solution. MATERIALS AND METHODS: 400 mL of whole blood were collected from 8 healthy volunteers, Plasma was removed by centrifugation separation method at 2,960g, 10 minute and replaced by 100 mL of SAG-M additive solution. Autologous transfusions were done in two groups on day 20 and 42 with Na51CrO4 (Dupont, USA) labelled RBCs. After 3, 10, 15, 20, 30 minutes and 1, 2, 24 hours, 20 days, the venous blood samples were collected from the volunteers, and their radioactivities were measured by the Gamma-counter (Cobra II, Germany), and 24 hour survival rate were calculated by Moroff's method. RESULTS: The mean 24 hour survival rate was 89.0% in 20 days preservation group and 71.8% in 42 days preservation group. The mean half life of transfused RBCs were 27.8 days (n=2)and 24.3 days (n=4) in 20 days and 42 days preservation group, respectively. No complications were observed during the study period. CONCLUSIONS: The RBCs preserved in SAG-M additive solution were near within allowable survival rates for transfusion.
Blood Preservation ; Centrifugation ; Erythrocytes* ; Half-Life ; Healthy Volunteers ; Korea ; Plasma ; Radioactivity ; Survival Rate ; Volunteers

We report a case in which routine chest roentgenograms of an 840 g infant led to the belief that the peripherally inserted central catheter (PICC) was appropriately positioned within the superior vena cava when, in actuality, it was within the azygous arch. Although many cases of pleural effusions have been reported to be caused by a central venous catheter, a right-sided hydrothorax caused by azygous vein rupture from the use of a PICC is an extremely rare complication. Sudden changes in the condition of a preterm infant with PICC should raise the suspicion of a catheter- related problem.
Azygos Vein* ; Catheterization, Central Venous ; Catheters* ; Central Venous Catheters ; Humans ; Hydrothorax* ; Infant ; Infant, Extremely Low Birth Weight* ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Pleural Effusion ; Rupture ; Thorax ; Veins ; Vena Cava, Superior

PURPOSE: It has been proposed that a decreased nephron number may be associated with the increased risk of glomerulosclerosis. In order to test the hypothesis that a reduced number and an increased volume of glomeruli may contribute to the pathogenesis of focal segmental glomerulosclerosis (FSGS), we compared the number and volume of glomeruli between 9 patients with FSGS and 8 with minimal change nephrotic syndrome (MCNS). METHODS: Mean glomerular volume was measured using the method of Weibel and Gomez. An estimate of glomerular number (index) was obtained by multiplying the cortical volume of a kidney by the fraction of renal cortex made up of glomeruli and dividing this by the mean glomerular volume for that kidney x 10(6). We determined kidney volume from ultrasonographic measurement. RESULTS: Patients with FSGS had significantly greater glomerular volume than patients with MCNS [2.02+/-0.36 (x10(6) micrometer3) vs. 1.57+/-0.27 (x10(6) micrometer3)] (p<0.025). However, there was no significant difference in the index of glomerular number (estimated glomerular number) between FSGS & MCNS patients (2.8+/-1.4 vs. 3.0+/-0.8). CONCLUSION: The glomerular volume was greater in FSGS patients than MCNS patients. But there was no significant difference in the index of glomerular number between patients with FSGS and MCNS.
Glomerulosclerosis, Focal Segmental* ; Humans ; Kidney ; Kidney Glomerulus ; Nephrons* ; Nephrosis, Lipoid* ; Nephrotic Syndrome

A splenic cystic lymphangioma is a very rare benign condition, and is classified as one of the cystic proliferations of the spleen. They are considered to result from developmental malformation of the lymphatic system and can be divided roughly into two types according to the extent of the disease: the isolated type, where only the spleen is involved, and the widespread type, where splenic involvement is an expression of multiple organ involvement. Thery are usually seen in children and often found incidentally. Herein, a case of cystic lymphangioma of the spleen in an elderly woman is presented, with emphasis on the rarity of cases in old age and on the problems of differential diagnosis in relation to other cystic proliferations of the spleen.
Aged* ; Child ; Diagnosis, Differential ; Female ; Humans ; Lymphangioma* ; Lymphangioma, Cystic ; Lymphatic System ; Spleen*

OBJECTIVES: This study aims to investigate the suitability of electronic health record (EHR) systems in Korea for global certification and to propose functions for future global systems by comparing and analyzing the certification criteria for Certification Commission for Health Information Technology (CCHIT) Certified Ambulatory EHR with BESTCare, which is the EHR system at Seoul National University Bundang hospital. METHODS: Domain expert groups were formed to analyze the inclusion of BESTCare functions and the types of differences for each of the CCHIT Certified 2011 Ambulatory EHR Certification Criteria. The types of differences were divided into differences in functions (F), differences in business processes (B), and differences in government policies (P). RESULTS: Generally, the criteria that showed differences in functions pertained to the connection between the diagnosis/problem list and order, the alert and warning functions for medication-diagnosis interactions, and the reminder/instruction/notification messages related to the patient's immunization status; these absent functions were enhanced clinical decision support system (CDSS) functions related to patient safety and healthcare quality. Differences in government policies were found in the pharmacy's electronic prescription functions, while differences in business processes were found in the functions constrained by the local workflow or internal policy, which require some customization. CONCLUSIONS: Functions that differed between the CCHIT certification criteria and the BESTCare system in this study should be considered when developing a global EHR system. Such a system will need to be easily customizable to adapt to various government policies and local business processes. These functions should be considered when developing a global EHR system certified by CCHIT in the future.
Certification ; Commerce ; Electronic Health Records ; Electronic Prescribing ; Immunization ; Korea ; Medical Informatics ; Patient Safety ; Quality of Health Care

Human sparganosis is a rare parasitic disease infected by plerocercoid larva (sparganum) of Spirometra species. It was usually diagnosed accidentally. In this article, we report a 44-year-old woman reanl allofraft recipient on cyclosporine and prednisolone as the immunosuppressive agents. She presented an enlarging subcutaneous nodule in the left thigh for 20 days, which was excised in the belief that it was a seroma. Characteristic sparganum lava accompanied by granulomatous inflammation and cyst formation in the subcutaneous tissue were discovered under microscopic examination of the excised tissue. However, the infectious source was not clear.
Adult ; Allografts* ; Cyclosporine ; Female ; Humans ; Immunosuppressive Agents ; Inflammation ; Larva ; Parasitic Diseases ; Prednisolone ; Seroma ; Sparganosis* ; Sparganum ; Spirometra ; Subcutaneous Tissue ; Thigh