Wiskott-Aldrich syndrome is a rare inheritable disease. Though there are many reported cases in the world, there are only 3 reported cases in Korea. We experienced a 7 years old boy with Wiskott-Aldrich syndrome. The initial presentation was generalized petechiae due to thrombocytopenia. We diagnosed him to have idiopathic thrombocytopenic purpura and followed up him for several years. During follow-up the patient have been suffered from croup, perianal abscess, bronchitis, and otitis media. One year ago, inguinal mass developed and 3 months ago, cervical and axillary masses developed. The fine needle aspiration cytology and biopsy revealed angioimmunoblastic lymphadenopathy like atypical lymphoproliferative disorders associated with congenital immune deficiency. So we diagonsed the patient to have Wiskott-Aldrich syndrome through his clinical course and laboratory findings including eczema, persistent low platelet counts, low mean platelet volume, monoclonal gammopathy on serum electrophoresis and biopsy finding.
Abscess ; Biopsy ; Biopsy, Fine-Needle ; Bronchitis ; Child ; Croup ; Eczema ; Electrophoresis ; Follow-Up Studies ; Humans ; Immunoblastic Lymphadenopathy ; Korea ; Lymphoproliferative Disorders ; Male ; Mean Platelet Volume ; Otitis Media ; Paraproteinemias ; Platelet Count ; Purpura ; Purpura, Thrombocytopenic, Idiopathic ; Thrombocytopenia* ; Wiskott-Aldrich Syndrome*

The purpose of this study was to verify the validity of the Patient Severity Classification Tool by examining the correlations between the APACHE III and the Patient Severity Classification Tool and to propose admission criteria to the ICU. The instruments used for this study were the APACHE III developed by Knaus and thePatient Severity Classification Tool developed by Korean Clinical Nurses Association. Data was collected from the 156 Medical ICU patients during their first 24 hours of admission at the Seoul National University Hospital by three trained Medical ICU nurses from April 20 to August 31 1999. Data were analyzed using the frequency, X2, Wilcoxon rank sum test, and Spearman rho. There was statistically significant correlations between the scores of the APACHE III and the Patient Severity Classification Tool. Mortality rate was increased as patients classification of severity in both the APACHE III and the Patient Severity Classification Tool scored higher. The Patient Severity Classification Tool was proved to be a valid and reliable tool, and a useful tool as one of the severity predicting factors, ICU admission criteria, information sharing between ICUs, quality evaluations of ICUs, and ICU nurse staffing. 1) This paper was awarded the first prize at the Seoul National Hospital Nursing Department Research Contest.
APACHE* ; Awards and Prizes ; Classification* ; Humans ; Information Dissemination ; Mortality ; Nursing ; Seoul

Oral or rectal sodium phosphate(NaP) preparation is commonly used for the colonic cleansing. Since the sole route of excretion of absorbed phosphate is renal, diminished renal function will limit the ability to excrete a sudden phosphate load. To date, about 20 adult cases of hyerphosphatemia associated with oral or rectal sodium phosphate solution have been reported, but not a single case in Korea to our knowledge. We report two cases of hyperphosphatemia and hypocalcemia after the administration of NaP(Colclean(R)) to reemphasizes the potential hazard of sodium phosphate bowel preparation in patients with compromised renal function.
Adult ; Colon ; Humans ; Hyperphosphatemia* ; Hypocalcemia* ; Kidney Failure, Chronic* ; Korea ; Laxatives* ; Sodium

A case of renal amyloidosis with crescentic glomerulonephritis associated with rheumatoid arthritis is described. A 60-year-old female with 15 years' history of rheumatoid arthritis developed nephrotic syndrome followed by rapid deterioration of renal function. Glomerular amyloid deposition and sclerotic change was present in kidney biopsy specimen and crescentic change was found in 85% of the glomerulus. Electron microscopic finding of glomerulus showed randomly oriented, rigid-appearing, long nonbranching fibrils. The patient was treated with intravenous high-dose methylprednisolone pulses combined with intravenous cyclophosphamide followed by oral corticosteroids. Three months after the above treatment, renal function gradually improved, reaching serum creatinine level to 2.5 mg/dL. But the nephrotic range proteinuria persisted.
Adrenal Cortex Hormones ; Amyloidosis* ; Arthritis, Rheumatoid* ; Biopsy ; Creatinine ; Cyclophosphamide ; Female ; Glomerulonephritis* ; Humans ; Kidney ; Methylprednisolone ; Middle Aged ; Nephrotic Syndrome ; Plaque, Amyloid ; Proteinuria

PURPOSE: Our aim was to evaluate the usefulness of fluid-attenuated inversion recovery (FLAIR) MR imaging for detection of acute subarachnoid hemorrhage (SAH) compared with unenhanced CT. MATERIALS AND METHODS: We compared FLAIR MR images with unenhanced CT scans in 28 patients with acute SAH. Findings of SAH on CT and MR images were graded as 0 (absence), 1 (suspicious), 2 (definite) in the cerebral sulci, sylvian fissure, basal cistern, and cisterns of the posterior fossa. We also compared FLAIR MR images of 28 patients with those of 35 normal subjects, and then the sensitivity, specificity, and diagnostic accuracy of FLAIR MR image for detection of acute SAH were calculated. RESULTS: FLAIR MR image was superior to CT in detecting SAH in the posterior fossa (1.41+/-.74 vs 0.78+/-.80; p<0.05) and cortical sulci(1.11+/-.80 vs 0.70+/-.83; p<0.05). There was no significant difference between FLAIR MR image and CT in detecting SAH in the basal cistern and sylvian fissure. The sensitivity, specificity, and diagnostic accuracy of FLAIR MR image for detection of SAH were 100% in all. CONCLUSION: FLAIR MR image is useful in detecting acute SAH, especially in patients with small amount of SAH or SAH in the posterior fossa.
Humans ; Magnetic Resonance Imaging* ; Sensitivity and Specificity ; Subarachnoid Hemorrhage* ; Tomography, X-Ray Computed

Ulcerative colitis is a chronic relapsing inflammatory disease localized on the colon. This disease is rare in children under 10 years of age. We experienced a case of ulcerative colitis in a 3-year-old male child. He was admitted due to fever, abdominal pain and diarrhea for 5 days, followed by rectal bleeding for 2 days. The diagnosis was established by clinical features, endoscopic and histologic findings. Taking combined medication of prednisolone and sulfasalazine, he achieved symptomatic remission and remained asymptomatic.
Abdominal Pain ; Child* ; Child, Preschool* ; Colitis, Ulcerative* ; Colon ; Diagnosis ; Diarrhea ; Fever ; Hemorrhage ; Humans ; Male ; Prednisolone ; Sulfasalazine ; Ulcer*

No abstract available.
Humans ; Listeria* ; Meningitis, Listeria*

OBJECTIVES: A study was conducted for investigating the status of simple X-rays on lumbosacral regions at pre-employment health examination and analysing the effectiveness of simple X-rays on lumbosacral regions. METHODS: The study data were pre-employment health examination data in a university hospital from Jan 3, 1993 through October of 1997. And, 97 newly employed workers who claimed no low back pain at pre-employment health examination of a certain company were follow-up survey about low back pain and related factors in April, 1995 and October of 1997. The data were collected by a self-reported questionnaire and a medical examination with a rehabilitation specialist. RESULTS: Taking the simple X-rays on lumbo-sacral regions was 1, 591 workers(10.5%) among 15,166 pre-employment health examination from Jan 3, 1993 through October of 1997. And yearly application rates of these X-ray tests have been significantly statistical increasing (p<0.01). 2. The abnormal findings of simple X-rays on lumbo-sacral regions at pre-employment health examination were 40 workers. But, there was not significantly statistical difference between normal and abnormal group for the complaint rate of low back pain. 3. The number of case with symptoms of low back pain at the follow-up survey were 46, so the incidence density was 37. 1 persons/ 100 person-years. And the incidence density was not significantly statistical difference between normal and abnormal group of simple X-rays on lumbo-sacral regions. 4. Through the multivariate logistic regression, significantly associated factors with low back pain were found to be tenure(OR=0.36, 95%CI: 0.17-0.79), lifting of heavy materials(OR=5. 86, 95%CI: l. 58-21. 74). CONCLUSION: The simple X-rays on lumbo-sacral regions at pre-employment health examination is required further research for utilizing pre-employment health examination according to above results.
Follow-Up Studies ; Incidence ; Lifting ; Logistic Models ; Low Back Pain ; Lumbosacral Region ; Questionnaires ; Rehabilitation ; Specialization

Truncus arteriosus is characterized by a single arterial vessel arising from the base of the heart and giving origin to the systemic, pulmonary and coronary circulation. To evaluate the incidence, types, and associated anomalies, 18cases of truncus arteriosus diagnosed by angiocardiography, were reviewed and were compared with other reports. Ten of them were confirmed by operation. The overall incidence of truncus arteriosus was 0.127%. Accordin to Collett and Edwards classification. Type I was encountered in 11cases(61%), type II in 4cases(22%), and type III in 3cases(17%), All the patients had subtruncal ventricular septal defects. Eight patients showed truncal valve regurgitation and valve stenosis was noted in one case. A right aortic arch was present in 9 cases. Associated anomalies were atrial septal defect(8cases), patent ductus arteriosus(3cases), coarctation of the aorta(2cases), isolation of the left subclavian artery(2 cases), and right aortic arch with an aberrant left subclavian artery(1case). Truncus arteriosus is an uncommon congenital cardio vascular malformation. In the group of cases which we encountered, type I was the most common anomaly. Frequently associated anomalies were right aortic arch, incompetent truncal valve and atrial septal defect.
Angiocardiography ; Aorta, Thoracic ; Classification ; Constriction, Pathologic ; Coronary Circulation ; Heart ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Incidence* ; Truncus Arteriosus* ; Vascular Malformations

BACKGROUND: Cyclosporine has a narrow therapeutic window and many serious side effects. The new oral microemulsion cyclosporine is known to have better absorption profile than non-microemulsion cyclosporine. The purpose of this study was to confirm above finding in stable renal transplant patients and also to compare correlation between AUC0-4 and C0, C2. METHODS: We checked the absorption profile of microemulsion cyclosporine group (N=15, ME group) and non-microeulsion cyclosporine group (N=15, NE group). All Patients had received renal transplantation at least 12 months before. Blood sampling for cyclosporine level was drawn before and at 1, 2, 3 hour after the cyclosporine morning dose (respectively C0, C1, C2 and C3). AUC0-4 was calculated with the formula: 256+C1+0.9xC2+1.4xC3. Age, sex, body weight, serum creatinine and cyclosporine dose were not different between ME group and NE group, but duration after transplantation was significantly higher in NE group (4.7+/-0.8 versus 3.0+/-1.9 year, p<0.05). RESULTS: AUC0-4 in ME group was significantly higher than NE group (2, 816+/-721 versus 2, 055+/-658 ng.h/mL, p<0.05). AUC0-4/dose, Cmax and Cmax/ dose were significantly higher in ME group. But these statistical differences were not consistent in both sexes. The difference of absorption profile between ME and NE group existed only in the female sex. In ME group, C1 correlated best with AUC0-4 (C0: r=0.493, C1: r=0.911, C2: r=0.906, C3: r= 0.789) and in NE group, C2 was the best (C0: r= 0.064, C1: r=0.958, C2: r=0.980, C3: r=0.912). CONCLUSION: Microemulsion cyclosporine is more bioavailable than non-microemulsion cyclosporine in stable renal transplant patients. C2 is better single time point marker for therapeutic drug monitoring in stable renal transplant patients than C0.
Absorption* ; Area Under Curve ; Body Weight ; Creatinine ; Cyclosporine* ; Drug Monitoring* ; Female ; Humans ; Kidney Transplantation ; Transplantation*