Pseudohypoaldosteronism type 1 is a genetic renal tubular disease of salt wasting, presenting in young infants. Tubular unresponsiveness to elevated endogenous and exogenous aldosterone is the suggested pathogenetic mechanism. Oral sodium chloride supplementation relieve the clinical symptoms and electrolyte distrubances. We experienced 2 cases of PHA type 1 in 38-day and 45-day old male infants who were presented with failure to thrive, vomiting and/or dehydration. Laboratory data showed hyponatremia, hyperkalemia, hypochloremia and metabolic acidosis. Renal and adrenal functions were normal. Plasma renin activity and plasma aldosterone concentration were markedly elevated. Under the diagnosis of pseudohypoaldosteronism type 1, oral supplementation of NaCl and/or kayexalate improved the clinical states of the patients.
Acidosis ; Aldosterone ; Dehydration ; Diagnosis ; Failure to Thrive ; Humans ; Hyperkalemia ; Hyponatremia ; Infant ; Male ; Plasma ; Pseudohypoaldosteronism* ; Renin ; Sodium Chloride ; Vomiting

No abstract available.
Hepatitis B* ; Hepatitis* ; Humans ; Infant, Newborn* ; Vaccination*

BACKGROUND: Laminin, which is present in all basement membrane, is a multifunctional glycoprotein with diverse biological activities. Like fibronectin, it can influence cell adhesion, growth, morphology, differentiation, and migration as well as the assembly of the extracellular matrix. Laminin primarily affects cells of epithelial origin, and response varies depending on the cells. OBJECTIVE: Studies about the relationship of laminin and the pathogenesis or metastasis of squamous cell carcinoma(SCC). MEHTODS: In this study, we have examined the laminin B1 mRNA expression and localizing pattern of laminin in SCC, using Northern and slot-blot hybridization, in situ hybridization and immunoperoxidase stain. RESULTS: In Northern blot hybridization, 5.6Kb mRNA transcript was detected in control and SCC specimens. Slot-blot hybridization indicated theat amounts of laminin B1 mRNA expression in SCC was 677.0+/-268.5 densitometer absorbance unit, in control was 228.0+/-16.37 unit respectively. There is a 3-fold increase in SCC compared to control. In in situ hybridization, 35S-labeled laminin B1 cDNA-mRNA hybrid granules were detected along the basement membrane and adjacent cells of SCC. In immunoperoxidase stain, laminin has stained strongly and continuously along the basement membrane of the tumor nest in well differentiated SCC, and weakly and interrupted in poorly differentiated SCC. CONCLUSION: These results suggested that laminin might be related to the pathogenesisand metastasis of squamous cell carcinomas.
Basement Membrane ; Blotting, Northern ; Carcinoma, Squamous Cell* ; Cell Adhesion ; Extracellular Matrix ; Fibronectins ; Glycoproteins ; In Situ Hybridization ; Laminin* ; Neoplasm Metastasis ; RNA, Messenger* ; Skin*

PURPOSE: The incidence of clinical nephritis is much higher especially in younger ages and in about one half of the cases, it also shows nephrotic syndrome. Thus, we examine the clinical and pathologic consideration of children with lupus nephritis and their treatment modality to improve the prognosis. MATERIAL AND METHOD: Among 67 cases of children under eighteen who were diagnosed SLE, 50 patients with hematuria and proteinuria from Jan. 1980 to Dec. 1996 were selected for the review. RESULTS: The ratio of the male to female patient was 1:3.5 and the average age at the diagnosis was 11.85+/-3.2 years old. Most common clinical manifestations at the time of the diagnosis were fever and skin rashes and the common laboratory results were proteinuria, hematuria, Out of 50 cases, 33 cases had renal biopsy. The results were 17 cases of Class IV, 7 cases of Class lll, 5 cases of Class lll, 3 cases of Class V and 1 case of Class l. Different treatment modalities were carried out; Corticosteroid only 21 cases, Corticosteroid+Azathioprine 25 cases, Corticosteroid+Cyclophosphamide 3 cases, and Corticosteroid+Cyclosporine A 1 case. However, there were no significant difference in the recurrence and complete remission rate of lupus nephritis in between each treatment groups. Average follow-up period was 37+/-23 months. Of all the follow-ups, 7 patients were dead. CONCLUSION: Early diagnosis should be carried out with renal biopsy, and should be considered for vigorous therapy, which currently includes high doses of corticosteroids and immunosuppressive drugs. Among these immunosuppressive agents, azathioprine has a lower incidence of long-term complications and low costs might be recommended. In addition, regular check-up for anti-DNA antibody, serum complement concentration and appropriate moniroting and management for the adverse effects of the treatment should enable to reach the continuous remission.
Adrenal Cortex Hormones ; Azathioprine* ; Biopsy ; Child* ; Complement System Proteins ; Diagnosis ; Early Diagnosis ; Exanthema ; Female ; Fever ; Follow-Up Studies ; Hematuria ; Humans ; Immunosuppressive Agents ; Incidence ; Lupus Nephritis* ; Male ; Nephritis ; Nephrotic Syndrome ; Prognosis ; Proteinuria ; Recurrence

The laparoscopic cholecystectomy, performed for the first time in France in 1987, has been accepted as in many countries the method of choice when performing a cholecystectomy. The trend toward minimally invasive surgery has prompted general surgeons to try it instead of open cholecystectomy. We performed 1000 laparoscopic cholecystectomies at the Department of General Surgery of Eulji Medical College Hospital from January 1992 to April 1997. The outcome data have been analyzed retrospectively. Moreover, we have compared the case histories of 13 patients who received cholecystostomy with those of 43 patient who did not. We also present preliminary results for an interval laparoscopic cholecystectomy which was a preceded by percutaneous cholecystostomy. The results are as follows: 1) The mean age was 54.3 years ranging from 17 to 86; there were 402 males and 598 females. 2) Of the 1000 patients, 123 patients (12.3%) had a history of abdominal surgery. 3) More than half of the patients(560 patients, 56.0%) underwent operations within 6 months of the onset of symptoms. 4) A preoperative endoscopic retrograde cholecystoscopy was performed on 220 patients (22.0%), and of these, 93 patients received endoscopic sphincterotomies because of bile duct stones. 5) The majority of patients(744 patients, 74.4%) had laparoscopic cholecystectomies which lasted 40 minutes or less. 6) More than half (54%) of the removed gallbladders showed grade II inflammation. 7) Drains were used in 142 patients (14.2%). 8) Postoperative complications were encountered in 48 patients (4.8%). 9) Postoperative oral intake was resumed within 24 hours in most patients. 10) Most patients(854 patients, 85.4%) were discharged within 3 days of the operation. 11) The pathologic diagnosis was chronic nonspecific inflammation in most cases (823 cases, 82.3%). 12) The most common microorganism in the bile was E. coli. 13) Patients who received a cholecystostomy had many benefits: a shorter operation time, a low rate of open cholecystectomy, early oral intake. 14) For the 1160 patients who underwent a cholecystectomy during the same period, the rate of open cholecystectomy was 13.8%.
Bile ; Bile Ducts ; Cholecystectomy ; Cholecystectomy, Laparoscopic* ; Cholecystostomy* ; Diagnosis ; Female ; France ; Gallbladder ; Humans ; Inflammation ; Male ; Postoperative Complications ; Retrospective Studies ; Sphincterotomy, Endoscopic ; Surgical Procedures, Minimally Invasive

PURPOSE:Idiopathic ketotic hypoglycemia (IKH) is an important cause of hypoglycemia in children. The purpose of this study was to determine the prevalence of ketotic hypoglycemia and to describe the clinical manifestation. METHODS:We conducted a retrospective chart review of children presenting hypoglycemia to the emergency department (ED) of Ewha Womans University Hospital between January 1995 and June 2004. Information recorded for subject included age, gender, weight, time of presentation, presenting symptoms, and laboratory data. RESULTS:Eighty two children were identified for hypoglycemia during the study period. IKH was the ultimate diagnosis for 66 patients (80.5%). Other diagnoses included hyperinsulinism (2.4%), drug adverse event (1.2%), sepsis (1.2%), and other disorder. The mean age for presentation of IKH was 37.9+/-18.6 months. Thirty eight boys and twenty eight girls were identified. The median time of presentation was 9:48 a.m. Of the 66 subjects, body weight of 46.9% patients was below 25th percentile for age. The average blood glucose was 41.4+/-14.2 mg/dL. Symptoms at presentation included lethargy (24.2%), mental change (16.7%) and vomiting (16.7%). 49 patients (74.2%) were described as having a concomitant illness. CONCLUSION: IKH was responsible for 80.4% of cases of hypoglycemia in pediatric ED and it had usually concomitant intercurrent illness which resulting in poor oral intake. Awareness of IKH, and its presenting characteristics, will be helpful in the ED when taking care of hypoglycemic children.
Blood Glucose ; Body Weight ; Child ; Diagnosis ; Emergency Service, Hospital ; Female ; Humans ; Hyperinsulinism ; Hypoglycemia* ; Lethargy ; Prevalence* ; Retrospective Studies ; Sepsis ; Vomiting

Serum is free of cellular components. Because DNA is located in the nuclei or mitochondria of cells, serum could be assumed DNA free. Few previously published case reports to date have used serum for DNA typing. Here, we report on human genotyping via short tandem repeat (STR) analysis using serum as a sample, and discuss problems involved in the process.
DNA Fingerprinting* ; DNA* ; Humans ; Microsatellite Repeats* ; Mitochondria

No abstract available.
Humans ; Plasma* ; Tranexamic Acid* ; Urticaria*

No abstract available.
B-Lymphocytes* ; Lymphoma, B-Cell* ; Waldenstrom Macroglobulinemia*

No abstract available.
Bowen's Disease*