We have experienced a case of infantile nephritic syndrome confirmed by renal biopsy in a 13-month-old female patient who showed and develop mental retardation and persistent proteinuria. She revealed mild eyelid edema, joint laxity, delayed speech development and adrenal cortical calcification on the radiologic study. Renal biopsy showed microcystic tubular change, micro-glomeruli and marked mesangial proliferation.
Biopsy ; Edema ; Eyelids ; Female ; Humans ; Infant ; Intellectual Disability ; Joint Instability ; Nephrotic Syndrome* ; Proteinuria

BACKGROUND: Left ventricular hypertrophy is a major cardiovascular risk factor for sudden death, acute myocardial infarction and congestive heart failure. The left ventricle is generally thought to adapt to sustained arterial hypertension with increased total peripheral resistance by developing concentric hypertrophy. In recent years, the echocardiogrphy has been developed as a noninvasive method for evaluation of left ventricular geometry and left ventricular mass. However, left ventricular adaptation to hypertension has been shown to be more complex than expected. In fact, many patients with mild to moderate hypertension exhibit normal left ventricular mass and wall thickness, other hypertensive patients have eccentric ventricular hypertrophy that is not related to systolic dysfunction, but rather to increased cardiac output and preload and in some hypertensive patients absolute and relative wall thickness is increased with normal ventricular mass(concentric remodeling). There are differences in the hemodynamics, systolic function and diastolic function in each group. METHODS: From september 1992 to August 1994, in 144 patients with untreated essential hypertension and 50 age and gender matched normal adults studied by two-dimensional, M-mode and Doppler echocardiography. In the present study we used echocardiographically derived left ventricular mass and relative wall thickness to assess the patterns of ventricular geometric adaptation to systemic hypertension and their relations to systemic hemodynamics, left ventricular load and contractile performance. RESULTS: Hypertensive group was 144 cases(M:F=68:76), the mean age 56+/-13years. Normotensive group was 50 cases(M:F=22:28), the mean age 52+/-9years. Among hypertensive patients, left ventricular mass index and relative wall thickness were normal in 42 cases(29%), 24 cases(17%) had increased relative wall thickness with normal ventricular mass(concentric remodelin),48 cases(33%) had both increased relative wall thickness and ventricular mass(concentric hypertrophy), 30 cases(21%) had increased left ventricular mass with normal relative wall thickness(eccentric hypertrophy). Concentric hypertrophy and normal left ventricle group are more common in untreated hypertensive patients in Korea. Systemic hemodynamics showed tendency to paralleled ventricular geomety. In groups with concentric remodeling and hypertrophy, perpheral resistance was increased. Cardiac index was midly increased in eccentric hypertrophy. Diastolic dysfunction was prominent tn concentric hypertrophy. CONCLUSION: Each patterns of left ventricular geometry had different systemic hemodynamics, ventricular pressure overload and ventricular volume overload. Therefore, appropriate selection of antihypertensive agent for the patients with each patterns of hypertrophy reduce the left ventricular hypertrophy and may improve the prognosis.
Adult ; Cardiac Output ; Death, Sudden ; Echocardiography ; Echocardiography, Doppler ; Heart Failure ; Heart Ventricles ; Hemodynamics ; Humans ; Hypertension* ; Hypertrophy ; Hypertrophy, Left Ventricular* ; Korea ; Myocardial Infarction ; Prognosis ; Risk Factors ; Vascular Resistance ; Ventricular Pressure

Hydroa vacciniforme is a rare, chronic photosensitivity disorder manifested in childhood by recurrent vesicles that hea1 with srarring. We report a case of hydroa vacciniforme in which vesicles were induced with multiple exposures ta UVA. The clinical features and ap-propriate laboratory evaluation of hydroa vacciniforme are reviewed.
Hydroa Vacciniforme ; Photosensitivity Disorders

The recent availability of recombinant human erythropoietin has opened new perspectives in the management of a variety of anemias. Clinical trials have been initiated in several countries using different approaches and methodology. We randomly assigned twelve premature infants(gestational age < 32 week) at high risk of requiring erythrocyte transfusion for anemia of prematurity with either subcutaneous recombinant human erythropoietin or a placebo. Treatment with rHuEPO was initiated at a dose of 100 units/kg day for 3 days a week. All patients were given supplemental oral iron therapy at a dose of 3 mg/kg per day, as tolerated and oral vitamin E at a dose of 25 units per day. Treated and control babies did not differ with respect to weight, hematocrit, overall mean reticulocyte count or rate of growth respectively. However, reticulocyte counts increased earlier in patients given rHuEPO. We conclude that rHuEPO administration is safe and feasible at the dose studied.
Anemia* ; Erythrocyte Transfusion ; Erythropoietin* ; Hematocrit ; Humans* ; Iron ; Pilot Projects* ; Reticulocyte Count ; Vitamin E ; Vitamins

No abstract available.
Iron*

No abstract available.

PURPOSE: To investigate the current situation and future trends in the field of refractive surgery in Korea. METHODS: Since 1995, the members of the Korean Society of Cataract and Refractive Surgery (KSCRS) have been annually surveyed on cataract and refractive surgery. The 2007 annual survey, consisting of 66 multiple-choice questions, was mailed in August 2008 to 336 KSCRS members. Fifty (14.9%) members answered. Current data were compared with those of previous annual survey and with those of the USA. RESULTS: The respondents in their forties accounted for the highest percentage (38%) of the subjects, and VISX S4 was the most popularly used (32%). The use of IntraLase femtosecond laser (AMO, USA) had increased. LASIK still accounted for the highest percentage (48%) of refractive surgeries. LASIK was preferred for myopia less than -8D, and phakic IOL was preferred for myopia more than -8D. Almost all the respondents answered that most promising refractive surgery is phakic IOL and the respondents who preferred LASIK decreased. CONCLUSIONS: This survey summarized current trends and changes in cataract and refractive surgery in Korea.
Cataract ; Axis, Cervical Vertebra ; Keratomileusis, Laser In Situ ; Korea ; Myopia ; Postal Service ; Refractive Surgical Procedures

Limb-body wall malformation complex(LBWC), also know as the amniotic band syndrome, is a poorly defined, sporadic group of congenital anomaly characterized by a collection of protean fetal malformation, deformation and disruption. Accurate diagnosis is often difficult because of its variable presentation pattern and the absence of exactly same case. We report two autopsy cases. One revealed body wall, cardiac, and craniofacial anomalies with anencephaly, and amniotic adhesive band attached to craniofacial defect of the fetal part without evidence of amniotic rupture. The other exibited abdominal wall defect with omphalocele, visceral, postural, and limb anomalies together with neural tube defect in the lumbosacral region. The pathogenesis of this syndrome was discussed in detail.

No abstract available.
Delivery of Health Care* ; Hearing Loss* ; Hearing* ; Noise*

We analyzed the MR findings of spinal epidural mass in 23 patients retrospectively. MR images were performed at 1.5T unit. The lesions were confirmed as metastasis(12 cases), lymphoma (2 cases), leukemia(1 cases), multiple myeloma(2 cases), meningioma(3 cases), neurofibroma(1 case), dysraphism with lipoma(1 case) and lipomatosis(4 cases), Most MRI examinations consisted of T1, proton density and T2 weighted sagittal imaging of the spine, with additional pulse sequences or image planes as needed for clarification. The level of the spinal epidural mass was cervical spine level in 2 cases, thoracic in 15 cases, lumbar in 7 cases, and sacral in 2 cases. The location of epidural mass within the spinal canal was eccentric in 20 cases, multiple in 2 cases, and encircled in 4 cases. Paraspinal mass was seen in 11 cases. Signal intensity of epidural mass was variable. The marrow of spine revealed low signal intensity(SI) on TIWI and high SI on T2WI in 14 cases. In conclusion, MRI is a useful diagnostic tool for the evaluation of spinal epidural mass.
Bone Marrow ; Humans ; Lymphoma ; Magnetic Resonance Imaging ; Protons ; Retrospective Studies ; Spinal Canal ; Spine