A 3-year-old-male had the appearance of red urine at birth and developed recurrent bullae in sun-exposed area of the skin, erythrodontia, alopecia, splenomegaly and hemolytic anemia, We observed coral red fluorescence of the teeth and urine under Wood's light and detected excessive excretion of the uroporphyrin in the urine and coproporphyrin in the stool wlth inreased porphyrin in the blood. Fluorescence of erythrocyte was demonstrated by:fluoreacence microscopy. Histologic findings showed subepidermal bulla with PAS-positive hyaline deposits around the blood vessels and revealed IgG deposits in the wall of blood vessels and dermo-epidermal junction by direct immunofluorescence.
Alopecia ; Anemia, Hemolytic ; Anthozoa ; Blood Vessels ; Erythrocytes ; Fluorescence ; Fluorescent Antibody Technique, Direct ; Hyalin ; Immunoglobulin G ; Microscopy ; Parturition ; Porphyria, Erythropoietic* ; Porphyrias ; Skin ; Splenomegaly ; Tooth

Holoprosencephaly is a rare and complex malformation affecting the cleavage of the developing forebrain and is usually associated with defects of the mid Face. We have experienced a case of holoprosencephaly, diagnosed prenatally by ultrasound examination at 31 weeks of pregnancy in a 31-year-old primigravida woman. This case is characterized by holoprosencephaly, cleft palate, cleft lip, left renal aplasia and right renal hypertrophy. The chromosomal study showed a deletion of the long arm of chromosome 7, 46, XX, del(7)(q32), We report with a terminal deletion of chromosome 7q associated with atypical clinical picture and holoprosencephaly.
Adult ; Arm ; Chromosome Aberrations* ; Chromosomes, Human, Pair 7 ; Cleft Lip ; Cleft Palate ; Female ; Holoprosencephaly ; Humans ; Hypertrophy ; Multicystic Dysplastic Kidney* ; Pregnancy ; Prosencephalon ; Ultrasonography

PURPOSE: The present study was designed to evaluate the incidence and characteristics of acquired cystic kidney disease (ACKD) in patients with end-stage renal disease who were treated by chronic hemodialysis, using renal ultrasonography. MATERIALS AND METHODS: We performed renal ultrasonography as a screening modality in 112 patients treated with long-term hemodialysis due to end-stage renal disease. We classified the patients years from one to six years. RESULTS: In our study, cystic lesions were noted in 41 patients (36.6%) and there was no sexual predilection of ACKD. Also, a prolonged period of hemodialysis increased the incidence of ACKD, especially for those treated more than 49 months. We encountered a case of renal cell cancer, evaluated it with CT, and then treated it by radical nephrectomy. CONCLUSIONS: With these results, renal ultrasonography can be used as a screening modality in patients treated with long-term hemodialysis more than 49 months due to end-stage renal disease. As complications occur, further evaluation will be needed.
Carcinoma, Renal Cell ; Humans ; Incidence ; Kidney Diseases, Cystic* ; Kidney Failure, Chronic ; Mass Screening ; Nephrectomy ; Renal Dialysis* ; Ultrasonography

Addisons disease is relatively rare than secondary adrenal insufficiency and result from progressive adrenocortical destruction. The common causes are idiopathic autoimmune atrophy and tuberculosis of adrenal glands. It is generally regarded as incurable in the sense that substitution therapy is required for the rest of the patients life. We report a case of tuberculous primary adrenal insufficiency which was confirmed by biopsy. This case was treated with antituberculous medication and showen to have improved adrenocortical function after six months.
Addison Disease* ; Adrenal Glands ; Adrenal Insufficiency ; Atrophy ; Biopsy ; Humans ; Tuberculosis

Objectives: This study aimed to determine whether collagen triple helix repeat containing-1 (CTHRC1), which is involved in vascular remodeling and bone formation, can stimulate odontogenic differentiation and angiogenesis when administered to human dental pulp stem cells (hDPSCs). Materials and Methods: The viability of hDPSCs upon exposure to CTHRC1 was assessed with the WST-1 assay. CTHRC1 doses of 5, 10, and 20 µg/mL were administered to hDPSCs.Reverse-transcription polymerase reaction was used to detect dentin sialophosphoprotein, dentin matrix protein 1, vascular endothelial growth factor, and fibroblast growth factor 2. The formation of mineralization nodules was evaluated using Alizarin red. A scratch wound assay was conducted to evaluate the effect of CTHRC1 on cell migration. Data were analyzed using 1-way analysis of variance followed by the Tukey post hoc test. The threshold for statistical significance was set at p < 0.05. Results: CTHRC1 doses of 5, 10, and 20 µg/mL had no significant effect on the viability of hDPSCs. Mineralized nodules were formed and odontogenic markers were upregulated, indicating that CTHRC1 promoted odontogenic differentiation. Scratch wound assays demonstrated that CTHRC1 significantly enhanced the migration of hDPSCs. Conclusions CTHRC1 promoted odontogenic differentiation and mineralization in hDPSCs.

BACKGROUND: The reference values of laboratory should review regularly to maintain good quality of practice. This is the second report of studies on reference values of Korea Association of Health Promotion (KAHP). The first one was reported in 2002 in Journal of Laboratory Medicine and Quality Assurance in Korea. The aim of this study is to know the changes of reference values for the past five years. METHODS: The way to analyze the data this time was essentially the same as the previous one (Indirect Method). The data from January to December, 2007 were collected. They totalled 5,133,327 test results from approximately 140 thousands individuals who visited for health checkup. The data were statistically analyzed with Minitab version 15.1.20.0 for Gaussian distribution using Anderson-Darling test. The trimming process repeated for the outliers, the results lying outside of +/-3SD, and as much as four times in certain test items, though, none of the tests showed Gaussian distribution. Subsequently, the reference values of most tests were defined in the ranges from the point of lowest 2.5% to the point of highest 97.5% and others were those, below 95 percentiles according to CLSI C28-A3 guideline. RESULTS: The reference ranges of 56 test items were either set as before or adjusted with new values, and compared. CONCLUSIONS: Comparing to the previous reference values (2002), the tests for liver function showed the lower upper values and the tests for diabetes and lipids showed higher upper values. Others were changed minimally with no significance.
Deception ; Health Promotion ; Korea ; Liver ; Normal Distribution ; Reference Values

PURPOSE: The purpose of this study was to explore changes in the test items in the preparation book for the national qualifying examination. Test items which developed in the year 2000 and 2004 through nationwide faculty workshop in Fundamentals of Nursing were examined. METHOD: Test items for the national qualifying examination for 2000 and 2004 in Fundamentals of Nursing were analyzed using frequencies and percent. RESULTS: The test items for the national qualifying examination for 2004 in Fundamentals of Nursing showed an increase in the number of test item over the year 2000. According to McGuire's taxonomy, domains of the test items in 2000 were recall(65.1%), interpretation (22.0%), and problem solving(12.9%) in that order. In 2004 items for the recall domain(74.7%) increased 9.6% above the level of 2000, and interpretation and problem solving domains showed a slight decrease. With regard to type of test items, the proportion of A type in 2004 was the same with 37.6% as 2000, and that of K type(61.5%) increased by 0.7% over the year 2000(60.8%). CONCLUSION: The test items for the national qualifying examination of 2000 and 2004 were not different except for the increase in the number of test items.
Classification ; Education ; Nursing* ; Problem Solving

OBJECTIVES: There is a increasing need to recruit and retain more psychiatrists who can plan and implement organized programs to work with chronic mentally ill patients in community settings. The aim of study is to discover what training is currently in place to prepare psychiatrist for work in "community" or "public" settings. METHODS: Survey questionnaires were mailed to 85 residency training directors and 10 leaders of community mental health in 2005. Response rate was 75%. RESULTS: Academic seminar about community mental health were not administered to residents in 63% of training hospitals. Forty one out of 64 training hospitals had community mental health rotation programs. Community mental health center (50%) and day hospital (36%) were the most common type of programs. Few general hospital had affiliation with community mental health programs. The amount of time for clinical rotation varied from less than three months part time to 6 months fulltime, with most frequent form of one day per week for three month in second or third year of psychiatry residents. CONCLUSION: Further steps are needed to improve residency training curricula and to encourage well qualified psychiatrist to choose careers in community psychiatry.
Community Psychiatry ; Curriculum ; Hospitals, General ; Humans ; Hypogonadism ; Internship and Residency ; Mental Health ; Mentally Ill Persons ; Mitochondrial Diseases ; Ophthalmoplegia ; Postal Service ; Psychiatry ; Surveys and Questionnaires

A 49-year-old male presented with a giant bullous emphysematous lung mimicking a pneumothorax. The initial chest X-ray revealed that the left lung was totally collapsed. A chest tube was inserted at the emergency room, after that an iatrogenic pneumothorax developed. The HRCT showed giant bullous emphysema mimicking a pneumothorax. The pneumothorax spontaneously resolved without special treatment. His bullous lung lesion had remained unchanged until last follow-up.
Chest Tubes ; Emergency Service, Hospital ; Emphysema* ; Follow-Up Studies ; Humans ; Lung ; Male ; Middle Aged ; Pneumothorax* ; Thorax*

The echinostome metacercariae encysted in Cipangopaludina sp. snails that were purchased from a market in Vientiane Municipality, Lao PDR, were identified as Echinostoma macrorchis (Digenea: Echinostomatidae) through recovery of adult flukes after experimental infection to rats and a cat. The metacercariae were round, 113-128 (121)x113-125 (120) microm, having a thick cyst wall, a head collar armed with collar spines, and excretory granules. The adult flukes recovered from the rats and cat at day 14 and 30 post-infection, respectively, were elongated, ventrally curved, and 3.9-6.3x0.7-1.1 mm in size. The head collar was distinct, bearing 43-45 collar spines with 5 angle spines on each side. Two testes were large (as the name implies), tandem, and slightly constricted at the middle, with irregular margins. Eggs were operculated, ovoid to elliptical, and 88-95x56-60 microm. In scanning electron microscopy, the head collar was prominent, with 43-45 collar spines. Scale-like tegumental spines were densely distributed on the ventral surface between the oral and ventral suckers. Sensory papillae were distributed mainly on the tegument around the 2 suckers. It is confirmed that E. macrorchis is distributed in Lao PDR using Cipangopaludina sp. snails as the second intermediate host.
Animal Experimentation ; Animal Structures/anatomy & histology ; Animals ; Biometry ; Cats ; Echinostoma/anatomy & histology/*isolation & purification ; Laos ; Microscopy/methods ; Parasitology/methods ; Rats ; Snails/*parasitology