Generalized pustular psorissis which is originary described by von Zumbusch in 1910, is the most intense form of the acute exanthematic variant among psoriasis and it is to be occured as a rare disease. We presented a case of generalized pustular psoriasis of a 16 year-old girl, who suddenly developed wide spread pustules during the acute exacerbating course of the psoriasis which had started from the primary plaque on her left anterior chest one month ago. She was free from subjective symptom. As the objective sign, the leukocytosis was only one of the significant laboratory findings. All skin lesions were almost eleared by the oral administration of methotrexate in 3 months.
Administration, Oral ; Adolescent ; Female ; Humans ; Leukocytosis ; Methotrexate ; Psoriasis* ; Rare Diseases ; Skin ; Thorax

Primary hyperparathyroidism is a rare endocrine disease in children. It involves bone and joint, urinary tract, gastrointestinal tract and cardiovascular system. The main cause of these involvement is high level of PTH in serum, resulting in hypercalcemia.An 11 years old male patient who had complained of limping gait since last 18 months, showed typical laboratory and radiological findings of primary hyperparathyroidism. At the ultrasonography, computed tomography and radionuclide scanning, a well defined mass(10 X 15mm) was found on the posterior aspect of the right thyroid lobe. The mass was confirmed histologically as adenoma of parathyroid gland. The patient was successfully treated with subtotal parathyroidectomy and temporal administration of calcium and vitamin D.We report this case of primary hyperparathyroidism with brief review of the literatures.
Adenoma ; Calcium ; Cardiovascular System ; Child ; Endocrine System Diseases ; Gait ; Gastrointestinal Tract ; Humans ; Hyperparathyroidism, Primary ; Joints ; Male ; Parathyroid Glands ; Parathyroidectomy ; Thyroid Gland ; Ultrasonography ; Urinary Tract ; Vitamins

The author observed clinical study and response of -interferon treatment of 43 cases of chronic hepatitis who were admitted to Department of Pediatrics Pusan National University College of Medicine during the period of 4 year 6 months from Jenuary, 1988 to Jun, 1992. The results were as follows: 1) Of 43 patients with chronic hepatitis, CPH was 17 cases (39.5%) and CAH was 26 cases (60.5%). 2) Of 43 patients, chronic hepatitis caused by HBV was 37 cases (86.0%) 2 cases were by Wilson's disease, 1 case was by glycogen storage disease and in the 3 cases, the cause was undetermined and CPH by HBV and CAH were 14 cases (82.4%), 23 cases (88.5%) respectively. 3) A peak frequency (46.5%) of chronic hepatitis occurred between 6 to age and male predominated in a propotion of 3.5:1. 4) On the clinical manifestation, jaundice and hepatomegaly was higher frequency on CAH than on CPH. 5) On the laboratory findings, CAH had higher serum ALT and direct bilirubin level than CPH. 6) On effect of -interferon treatment for patients of CAH, serum ALT and AST levels normalized in 81.8% of patients, negative conversion of HBeAg was 38.5%, Anti-HBe serocoversion was 7.7%, negative conversion of HBsAg was 7.7% and the clearnce of HBV-DNA from seum showed 60.0%. 7) The HBeAg seroconversion (28.6%) on children of HBsAg(+) mother showed lower reponse than HBeAg seroconversion (50.0%) of HBsAg(-) mother after -interferon treatment for patients of CAH.
Bilirubin ; Busan ; Child* ; Glycogen Storage Disease ; Hepatitis B e Antigens ; Hepatitis B Surface Antigens ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Hepatolenticular Degeneration ; Hepatomegaly ; Humans ; Jaundice ; Male ; Mothers ; Pediatrics

No abstract available.
Bone Density* ; Female ; Lactation*

No abstract available.
Bone Density* ; Female ; Lactation*

Ehlers-Danlos Syndrome(EDS) is an inherited disorder of the connective tissue disease that is usually transmitted as autosomal dominant trait. The defect in the biogenesis of collagen results in varying degree of skin hyperextensibility, joint hypermobility, skin fragility and bruising. There have been several reports of cardiac or great vessel abnormalities in patients with the EDS. A 45-year-old Woman was admitted to Maryknoll hospital because of orthopnea, palpitation and epigastric discomfort. Physical examination reveals multiple variable sized bruises and increased hyperelasticity on skin, and hypermobile joint in knee and proximal interphalanges of both hands. Mitral valvular prolapse and atrial septal defect were detected by echocardiography. We report a case of EDS accompanied by mitral valvular prolapse and ostium secundum atral septal defect with brief review of literature.
Organelle Biogenesis ; Collagen ; Connective Tissue Diseases ; Contusions ; Echocardiography ; Ehlers-Danlos Syndrome* ; Female ; Hand ; Heart Septal Defects, Atrial* ; Humans ; Joint Instability ; Joints ; Knee ; Middle Aged ; Physical Examination ; Prolapse* ; Skin

PURPOSE: In conservative treatment of burn wounds, early epithelialization is essential to obtain good aesthetic and functional results. When choosing of dressing method to treat burn wound we should consider an optimal environment for healing. In this study, we used enzyme alginogel that is a new class of non cytotoxic wound care products. METHODS: Patient information from May 2012 to October 2012 was analysed for degree of epithelialization, bacterial loads and pain. We retrospectively reviewed the efficacy of enzyme alginogel (Flaminal(R)) in the experimenal group of 31 patients treated with this product and compared this with a control group of 30 patients treated with classical treatment. RESULTS: The study showed a significantly shorter healing time and decresed pain in the Flaminal(R) group. A limited number of patients were found to be positive for wound swabs and no severe inflammation and allergic reaction. CONCLUSION: Applying enzyme alginogel (Flaminal(R)) to burn wound showed satisfactory results in epithelialization, degree of pain. It can be one of treatment options for partial thickness burn wound.
Bacterial Load ; Bandages ; Burns* ; Humans ; Hypersensitivity ; Inflammation ; Methods ; Retrospective Studies ; Wounds and Injuries

Nephrotoxicity is a major limiting factor in the use of aminoglycoside antibiotics, the mechanisms for which are still speculative. To clarify the mechanisms of renal tubular cell death induced by aminoglycosides, we examined the renal proximal tubule-like cell line, LLC-PK1, after inducing apoptosis through a chronic treatment with gentamicin (GM). Changes in the expression of the Fas were also investigated. On flow cytometric analysis, 5.7 +/- 3.3% of the control cells appeared in a region of decreased forward light scatter and increased side light scatter, where both indices represent the characteristics of apoptotic cell death. Compared to the control, treatment with 10 mM of GM for 15 days significantly increased the proportion of cells in the apoptotic region to 23.9 +/- 8.5%. This finding was supported by electrophoretic analysis of the DNA extracted from the GM-treated cells, where a series of bands corresponding to integer multiples of 180 to 200 base pairs was visualized. However, the 15-day GM treatment did not cause a significant elevation in the expression of the 45 kD Fas protein, the cell surface molecule that stimulates apoptosis, by Western blot analysis. In conclusion, long-term exposure to GM induces apoptosis of the renal tubular epithelial cells, and this process may contribute to some of the aminoglycoside nephrotoxicities. Further studies are needed on the mechanism(s) of apoptosis induced by GM.
Animal ; Antibiotics, Aminoglycoside/toxicity* ; Antigens, CD95/analysis ; Apoptosis/drug effects* ; Cell Line ; Gentamicins/toxicity* ; Kidney Tubules, Proximal/pathology ; Kidney Tubules, Proximal/drug effects* ; Swine

Hemolytic uremic syndrome (HUS) is a rare disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. These characteristics are related to the formation of platelet-rich microthrombi in the microvasculature. HUS is associated with a variety of etiologies, including cisplatin. Previously reported HUS cases after cisplatin administration were almost always related to systemic combination chemotherapy that included cisplatin. Transarterial chemoembolization (TACE) is commonly used in treatment of hepatocellular carcinoma, and most centers in Korea use cisplatin. A 70-year-old female with hepatocellular carcinoma was treated with TACE including cisplatin and subsequently developed clinical and laboratory findings compatible with HUS.
Acute Kidney Injury ; Anemia, Hemolytic ; Carcinoma, Hepatocellular ; Chemoembolization, Therapeutic ; Cisplatin ; Drug Therapy, Combination ; Female ; Hemolytic-Uremic Syndrome ; Humans ; Korea ; Microvessels ; Thrombocytopenia

BACKGROUND: It has been well known that parathyroid hormone (PTH) plays an important role as a uremic toxin in patients with end-stage renal disease, and develops many abnormalities in musculo-skeletal, nervous, cardiovascular and immune systems, which contributes mortalities and morbidities of the patients. Recently, many researches have reported various clinical significances of PTH. Especially, there is some suggestion that parathyroid hormone level could be used as a new marker of survival in uremic patients. The purpose of this study was to assess the prevalence of secondary hyperparathyroidism according to the modality and duration of dialysis in patients with ESRD and to determine the factors that attribute to increasing iPTH levels. METHODS: From January 1996 to June 2001, 328 dialysis patients who have received maintenance hemodialysis or peritoneal dialysis more than 6 months in Severance Hospital were included in this study. We studied retrospectively by reviewing their medical records and defined hyperparathyroidism, if iPTH level was increased more than three times of upper normal limit. RESULTS: The number of patients with iPTH level greater than 195 pg/mL was 170(49.8%): HD 76 (53.9%), CAPD 94 (50.2%). The average level of iPTH was increased with dialysis duration in both groups, and also, significantly more increased in hemodialysis group than CAPD more than 40months after the beginning of dialysis. The iPTH level was negatively correlated with age and creatinine clearance by simple and multiple regression analysis in both dialysis groups. Alkaline phosphatase and serum phosphorus levels had positive correlation with the level of iPTH in both CAPD and HD patients. Cox regression analysis showed an increment of development of secondary hyperparathyroidism with duration of dialysis. In patients on HD, the prevalence of secondary hyperparathyroidism was much higher than those on CAPD with statistical significance in patients on dialysis more than 40 months. CONCLUSION: In this study, iPTH level was correlated with age, serum phosphorus level and residual renal function at the initiation of dialysis in both groups. And also, in patients on HD, the average level of iPTH and the prevalence of secondary hyperparathyroidism were much higher than those on CAPD with statistical significance. So, we suggest that early dialysis and intensive attention to serum phosphorus level would be required in patients with ESRD for preventing the secondary hyperparathyroidism and more careful monitoring for serum calcium, phosphorus and parathyroid hormone with adequate treatments would be needed especially in hemodialysis patients.
Alkaline Phosphatase ; Calcium ; Creatinine ; Dialysis* ; Humans ; Hyperparathyroidism ; Hyperparathyroidism, Secondary ; Immune System ; Kidney Failure, Chronic* ; Medical Records ; Mortality ; Parathyroid Hormone ; Peritoneal Dialysis ; Peritoneal Dialysis, Continuous Ambulatory ; Phosphorus ; Prevalence ; Renal Dialysis ; Retrospective Studies