Few cases of ichthyosis bullosa of Siemens(IBS) have been reported since 1939, as a distinct entity from bullous congenital ichthyosiform erythroderma(BCIE). IBS can be differentiated from BCIE by the absence of congenital erythroderma and a different distribution of involved skin area. It's characteristic features include blistering, superficial erosion or moulting of the outer skin. Histological features are tonofilaments aggregation confined to the granular and upper spinous layer of the epidermis. However, in BCIE these findings are present in the whole suprabasal compartment. The original reports of Siemens and cases from other authors showed an autosomal dominant inheritance. Our patient developed IBS sporadically without a familial background.
Blister ; Dermatitis, Exfoliative ; Epidermis ; Humans ; Ichthyosis Bullosa of Siemens* ; Ichthyosis* ; Intermediate Filaments ; Molting ; Skin ; Wills

The abnormalities in Hypothalamic-pituitary-adrenal(HPA) axis are associated with many psychiatric symptoms including depression. We present a report of a 71 year old man who was admitted to the psychiatric department presenting symptoms of headache, avolition, loss of energy, psychomotor retardation, poor appetite, insomnia, anxiety resulting from adrenal insufficiency and hypopituitarism. Hypothyroidism and electrolyte disturbance were managed and headache, insomnia, anxiety, GI symptoms were improved. But he remained in anergic state. After discharge, he was readmitted to infection department with high fever and drowsy mentality. Adrenal insufficiency was recognized and he was treated with corticosteroid replacement therapy. Finally his diagnosis was made as panhypopituitarism and overall symptoms were resolved. In this case, we showed how the atypical symptoms resulting from hypopituitarism develop and progress. Hypothyroidism, adrenal insufficiency, and growth hormone deficiency resulting secondarily from panhypopituitarism were associated with various nonspecific symptoms such as loss of energy, fatigue, insomnia, weight loss, decreased appetite etc. In clinical situation, differential diagnosis with depression is needed when clinicians were met a patient with these nonspecific symptoms. It is important that laboratory tests and differential diagnosis with endocrine diseases should be conducted, especially in geriatric patients with nonspecific symptoms like anergia, fatigue, poor appetite and so on.
Adrenal Insufficiency ; Anxiety ; Appetite ; Axis ; Depression ; Diagnosis, Differential ; Endocrine System Diseases ; Fatigue ; Fever ; Growth Hormone ; Headache ; Humans ; Hypopituitarism ; Hypothyroidism ; Sleep Initiation and Maintenance Disorders ; Weight Loss

PURPOSE: We evaluated the frequency of detection of small hepatic cystic lesion(SHC) on abdominal CT films and its significance in patients with extrahepatic malignancies. METHODS AND MATERIALS: We retrospectively reviewed the abdominal CT films of a total 1112 patients to determine the frequency of detection of SHC, and 62 patients who had both extrahepatic malignancies and SHC were selected. A total 106 SHC determined as benign or malignant in 40 patients was analyzed according to the size(< 1 cm, 1 2cm), the number(1, 2 4, >4), the margin(well-defined, iil-defined),and the location(central, peripheral) of individual cystic lesions. The findings of follow-up CT and ultrasonography and the results of biopsy were also reviewed. The frequency of combined renal cyst was also considered. RESULT: SHC of those 62 patients were classified as benign(27 patients, 43.6%), malignant(13 patients, 20. 9%), or undetermined(22 patients, 35.5%) by the follow-up CT, ultrasonography, and biopsy. Most benign SHC (74.4%) measured under 1 cm, but 75% of the malignant SHC were in 1 -2cm in size. The percentage of the malignancy in one and two to four SHC was 37.5% and 40%, respectively. The margin of SHC was ill-defined in 67.9% of the benign and 64.3% of the malignant lesions. SHCs were mainly located at the periphery of the liver(benign :62.7%, malignant: 64.3%). Coexisting renal cyst was rare. CONCLUSION: SHC is not uncommon finding(13.2%) in patients with extrahepatic malignancy and has a high probablity of metastasis when it is larger than 1 cm, which should be closely followed and intensively studied further.
Biopsy ; Follow-Up Studies ; Humans ; Incidence* ; Neoplasm Metastasis ; Retrospective Studies ; Tomography, X-Ray Computed ; Ultrasonography

PURPOSE: To construct new Korean reference curves for birth weight by sex and gestational age using contemporary Korean birth weight data and to compare them with the Lubchenco and the 2010 United States (US) intrauterine growth curves. METHODS: Data of 2,336,727 newborns by the Korean Statistical Information Service (2008-2012) were used. Smoothed percentile curves were created by the Lambda Mu Sigma method using subsample of singleton. The new Korean reference curves were compared with the Lubchenco and the 2010 US intrauterine growth curves. RESULTS: Reference of the 3rd, 10th, 25th, 50th, 75th, 90th, and 97th percentiles birth weight by gestational age were made using 2,249,804 (male, 1,159,070) singleton newborns with gestational age 23-43 weeks. Separate birth weight curves were constructed for male and female. The Korean reference curves are similar to the 2010 US intrauterine growth curves. However, the cutoff values for small for gestational age (<10th percentile) of the new Korean curves differed from those of the Lubchenco curves for each gestational age. The Lubchenco curves underestimated the percentage of infants who were born small for gestational age. CONCLUSION: The new Korean reference curves for birth weight show a different pattern from the Lubchenco curves, which were made from white neonates more than 60 years ago. Further research on short-term and long-term health outcomes of small for gestational age babies based on the new Korean reference data is needed.
Asian Continental Ancestry Group ; Birth Weight* ; Female ; Gestational Age* ; Growth Charts ; Humans ; Infant ; Infant, Newborn ; Information Services* ; Male ; United States

PURPOSE: The staging of bladder carcinoma is a major determinant of operative management. CT of bladder carcinoma has been widely used to diagnose external extension (pT3b and over), but tumors confined to the bladder wall (from pT1 to pT3a) are poorly delineated. The authors describe CT findings of the superficial bladder carcinoma (below T1, stage A) in thin section CT with air insufflation technique (air insufflation-CT) to facilitate early detection and to aid correct staging of the superficial bladder carcinoma. MATERIALS AND METHODS: The materials consisted of proved 24 cases (1'9 patients, single tumor: 16 patients, multiple tumors :3 patients) of stage A bladder carcinomas. Air insufflation-CT was performed by the infusion of approximately 200 mL of air into the bladder via a Foley catheter. After the routine pelvic CT, bladder tumors were re-scanned with 1.5 to 5 mm thickness and intervals. RESULTS: The superficial bladder carcinomas were detected as nodular(5 cases, 20.8%), papillary(15 cases, 62.5%), pyramidal(2 cases, 8.3%), and domed(2 cases, 8.3%) forms on air insufflation CT. These tumors were classified into three types according to the size of the tumoral neck:type I(pedundulated polypold tumor:4 cases, 16.6%), type II(polypid tumor with short neck :13 cases, 54.2%), and type Ill(sessile tumor :7 cases, 29. 2%). The mean size(tumoral width x height x base o, neck/stalk) of the tumors was 22 x 20 x 16mm. The average tumoral sizes according to each type of the superficial tumors were type 1:22 x 25 x 6mm, type 11:23 x 22 x 18mm, and type III :18 x 15 x 18mm. The mean width of the type I--II tumoral necks was 15mm. The mean length of the type I tumoral neck(pedicle) was 2.5mm. Papillary fronds of the tumors were seen in 10 cases(41.7%) of 24 superficial tumors. Outer margin of the involved bladder wall was smooth in all cases. CONCLUSION: Thin-sectin CT with air insufflation technique for bladder carcinoma was useful in tumoral demonstration, and characteristics of the superficial bladder carcinomas were small polypold tumors had a short neck mostly and smooth outer wall of the involved bladder wall.
Catheters ; Humans ; Insufflation* ; Neck ; Urinary Bladder Neoplasms ; Urinary Bladder*

PURPOSE: Transcatheter arterial chemoembolization (TACE) and subsequent percutaneous ethanol injection (PEi) was attempted in 8 patients with 9 hepatocellular carcinomas (HCCs) for complete tumor necrosis of HCCs less than 5cm in greatest diameter. MATERIALS AND METHODS: PEI was performed with 2-8ml of absolute (99.9%) ethanol two weeks after TACE under CT or ultrasound guidance. For each patient PEI was done twice to four times within 4-10 days of each procedure. After completion of a series of PEI, follow up examination (range:3 months-l.5 year period) was done with angiography, CT or ultrasound and correlated with serum alpha-fetoprotein (AFP) level. RESULTS: On follow up anglograms, the lesions completely disappeared or decreased in size without tumor vessels or staining in 5 of 6 patients. On follow up CT of 6 patients, the lipiodol-laden HCCs were surrounded by non-enhancing low density and the losion sizes were slightly decreased or not changed. These are suggestive of necrosis of tumor itself and adjacent liver parenchyma. The tumors could not be detected on follow up ultrasound examination in 2 patients. Serum AFP was decreased in 7 patients and was well corresponded to the results of imaging modalities. CONCLUSION: The authors concluded that the combined TACE and PEI is an appropriate treatment for small HCCs having high surgical risks.
alpha-Fetoproteins ; Angiography ; Carcinoma, Hepatocellular* ; Ethanol* ; Follow-Up Studies ; Humans ; Liver ; Necrosis ; Ultrasonography

No abstract available.
Humans ; von Willebrand Diseases*

No abstract available.
Humans ; von Willebrand Diseases*

Systemic lupus erythematosus(SLE) is a disease of unknown etiology in which multiple organs are damaged by pathogenic autoantibodies and immune complexes. Neuropsychiatric manifestations in SLE were first described by Kaposi in 1872. These are so diverse that they include psychosis, depression, stroke, seizure and cognitive dysfunction etc. These patients are frequently consulted for psychiatric evaluation. Neuropsychiatric manifestations in SLE are also among the leading causes of morbidity and mortality and associated with poor long-term outcome. So it is essential to recognize and intervene these symptoms early. But the clear diagnostic criteria for CNS involvement in SLE have not been formulated, and diversity and fluctuation of illness make it difficult to confirm it. The authors reported three cases of SLE with severe neuropsychiatric manifestations. These patients showed symptoms such as disorientation, auditory and visual hallucibation, delusion and mood instability. They became frequently impulsive and violent and had risks to injure themselves or others. Although CNS involvement in SLE is not well known, we reviewed the pathogenesis, classification, diagnosis, clinical manifestation and treatment of them.
Antigen-Antibody Complex ; Autoantibodies ; Classification ; Delusions ; Depression ; Diagnosis ; Humans ; Lupus Erythematosus, Systemic* ; Mortality ; Psychotic Disorders ; Seizures ; Stroke

BACKGROUND: Cytokine response changes with ageing. CD26 is known as an operational marker for Th1 pathway. In this study we investigated the possibility of CD26 as a practical marker for immunosenescence, Th1 or Th2 polarization. METHODS: We studied 99 subjects from 4 groups divided by age (thirties and sixties) and by hepatitis B. CD26 expression in T cells in peripheral blood was investigated by 3 color flow cytometry. Especially, CD26 expression in activated (HLA-DR+) T cells was compared among each group. RESULTS: CD26 expression in total T cells had the trend toward a decrease with ageing and decreased significantly especially in Ts cells (from 45.9+/-11.3% to 37.5+/-12.4%, P<0.05). Regardless of hepatitis B, CD26 expression in activated Th cells was not significantly different between the two age groups. HLA-DR expression in total T cells had the trend toward an increase in the elderly or hepatitis B and the dominant T cell subset responsible for this increase was Ts cell subset in the elderly and was Th cell subset in hepatitis B. CONCLUSIONS: We could not find the possibility of CD26 as a practical marker for immunosenescence reflecting Th1 polarization in activated Th cells. But CD26 or HLA-DR expression in total T cells had the trend to change with ageing and the change in Ts cells was more dominant than in Th cells.
Aged ; Flow Cytometry ; Hepatitis B ; HLA-DR Antigens ; Humans ; T-Lymphocytes*