There had been total 20 patients with early onset(4 months~2 years) primary nephrotic syndrome in the Department of Pediatrics, Seoul National University Children's Hospital, during the period from March 1987 to February 1993. We analysed clinical courses, response to treatment, pathological findings and prognosis of the patients And the results were as follows; 1) The initial responders to steroid treatment were 10(50%), of whom 3 became late nonresponders. Of the 10 initial nonresponders, 8 revealed continuing nonresponsiveness. 2) Incidence of hypertension was significantly higher in the intial nonresponders than in the initial responders. 3) Renal biopsies were performed in 9 initial nonresponders and 2 late nonresponders. And the results were 6 with minimal change lesion, 4 with focal segmental glomerulosclerosis and 1 with mesangiocapillary glomerulonephritis. 4) Six continuing or late nonresponders received methylprednisolone pulse therapy or cyclophosphamide. And partial remission was induced in 4 of them. 5) The overall 3 year maintenance rate of normal renal function was 81%(100% in the initial responders and 64% in the initial nonresponders). In conclusion, the initial steroid responsiveness and prognosis were poorer in patients with early onset nephrotic syndrome than in patients with usual childhood nephrotic syndrome.
Biopsy ; Cyclophosphamide ; Glomerulonephritis, Membranoproliferative ; Glomerulosclerosis, Focal Segmental ; Humans ; Hypertension ; Incidence ; Methylprednisolone ; Nephrotic Syndrome* ; Pediatrics ; Prognosis ; Seoul

PURPOSE: We evaluated the capability of MR in the diagnosis of anomalous pulmonary venous connection (APVC). MATERIALS AND METHODS: The patient group consisted of 11 total APVC and 8 partial APVC diagnosed with MR. Echocardiography was performed in all cases, cardiac angiography in 12 cases and operation in 12 cases. We compared MR findings with those of operation, echocard iography and cardiac angiography. RESULTS: In surgically proven 12 cases, diagnostic accuracy of preoperative MR, echocardiography and cardiac angiography was 100%, 67% and 63%, respectively. In the remaining cases, MR findings well correlated with those of echocardiography or cardiac angiography. Stenosis of common pulmonary vein or superior vena cava was identified in 4 cases. In one patient, MR depicted associated cor triatriatum clearly. CONCLUSION: MR is an effective modallty in depicting anomalous pulmonary venous con nections.
Angiography ; Constriction, Pathologic ; Cor Triatriatum ; Diagnosis ; Echocardiography ; Humans ; Pulmonary Veins ; Vena Cava, Superior

No abstract available.
Female ; Humans ; Osteoporosis* ; Uterine Prolapse*

No abstract available.
Aneurysm* ; Heart Septal Defects, Ventricular*

To evaluate the differentiation status of smooth muscle in gastric stromal tumors which were negative for S-100 protein, immunohistochemistry using desmin, actin, myosin and vimentin was performed in 14 cases of gastric smooth muscle tumors. Ultrastructural Examination was also performed. For comparison a case of leiomyoma of the esophagus, a case of the sigmoid colon, 10 cases of the uterus were also examined. The results obtained were as follows. All gastric smooth muscle tumors showed vimentin-positivity. Six of 14 gastric smooth muscle tumors, (5 of 8 leiomyoma and 1 of 4 leiomyosarcoma) showed positivity for desmin, actin, and myosin(42.9%). All esophageal, colonic, and uterine leiomyomas showed diffuse positive reaction for desmin, actin, and myosin. Vimentin positivity was also noted in leiomyoma of the colon and uterus. Ultrastructurally, a few cells in the gastric stromal tumors had scattered microfilaments with dense bodies, subplasmalemmal dense plaques, and micropinocytic vesicles. However, most of the tumor cells did not have any of the ultrastructural features of smooth muscle differentiation. Leiomyomas of the esophagus and uterus showed many cytoplasmic microfilaments with dense bodies. These results suggest that most of the benign and malignant tumor cells of gastric stromal tumors have features of the undifferentiated cells, immunohistochemically as well as ultrastructurally, although a few cells have. It is speculated that most gastric stromal tumors may have lost their smooth muscle differentiation.

Fibrohistiocytic tumors are a diverse group of benign and malignant soft tissue lesions, including dermatofibroma, dermatofibrosarcomaprotuberans, and malignant fibrous histiocytoma. On the clinical point of view, the distinction between benign and malignant lesions and malignancy grading is far more important. Therefore, we investigated 23 fibrohistiocytic tumors, using PCNA (PC10) which was a useful marker of proliferating activity, to differentiate the benign lesions from the malignant and correlate with other prognostic factors including tumor necrosis. cellularity, histologic grade, and mitotic counts. The results obtained were as follows 1) Positive tumor cells were clearly identified by the characteristic diffuse or granular nuclear staining. 2) The number of PCNA-positive tumor cells were 2.16+/-2.39% in dermatofibroma, 16.12+/-7.38% in dermatofibrosacoma protuberans, and 28.02+/-17.47% in the malignant fibrous histiocytoma. The numbers of PCNA-positive tumor cells in the malignant lesions higher than in the benign (p<0.001). 3) Deep seated, large size (>5 cm) and recurred or metastatic cases of MFH were more the high PCNA index (more than 20%) than the low index (less than 20%) groups. 4) PCNA index in MFHs had positive correlation with the number of mitotic counts (r=0.7582, p<0.001), cellularity (r=0.5908, p<0.05) and histologic grade (r=0.4164, p<0.05). These results suggested that reactivity on PCNA might assist in the distinction between benign and malignant lesions in fibrohistiocytic tumors, and could be a useful prognostic factor in the patients with malignant fibrous histiocytoma.
Neoplasm Metastasis

The clinical data of 27 patiets with hypophosphatemic rickets treated with phosphate and 1alpha-hydroxyvitamin D were analysed retrospectively. The median age at diagnosis was 4 years, and the main clinical manifestations were bowleg and short stature. Among total 24 families, 5 families (21%) had X-linked dominant mode of inheritance, 1 family (4%) had autosomal dominant mode and 17 families (71%) had no family history, The serum phosphorus concentration rose from initial value of 2.7+/-0.13mg/dl to 3.5+/-0.19mg/dl. The serum alkaline phosphatase was reduced from 871+/-63IU/L to 393+/-41IU/L. Healing of rickets was demonstrated by radiography. Patients treated for at least two years before the onset of puberty had an increase in the mean height SD score from -1.58 to -0.79. Orthopedic surgeries for severe lower extremity deformity were performed in 11 patients, of whom 8(73%) were dignosed over 5 years of age. Complications of therapy were as follows; 12 patients (44%) developed more than one episode of hypercalciuria, 5 patients (19%) developed more than one episode of hypercalcemia, and nephrocalcinosis was noted in 5(33%) out of 15 patients by renal ultrasound. The group with nephrocalcinosis had a higher incidence of hypercalemic episodes than the group without nephrocalcinosis. In conclusion, treatment of hypophosphatemic rickets results in healing of rickets and acceleration of growth, and we must evaluate complications of therapy such as hypercalcemia and nephrocalcinosis.
Acceleration ; Adolescent ; Alkaline Phosphatase ; Congenital Abnormalities ; Diagnosis ; Humans ; Hypercalcemia ; Hypercalciuria ; Incidence ; Lower Extremity ; Nephrocalcinosis ; Orthopedics ; Phosphorus ; Puberty ; Radiography ; Retrospective Studies ; Rickets ; Rickets, Hypophosphatemic* ; Ultrasonography ; Wills

We reviewed the clinical and hemodynamic studies in 10 patients with the isolated congenital mitral insufficiency. The patients ranged in age from 6 months to 16 years at the time of diagnosis and the incidence was 0.26% among the total congenital heart disease proven by cardiac catheterization and angiography. In two cases anterior mitral cleft was demonstrated by 2-D echocardiography and/or operation. In two cases left coronary artery was originated from pulmonary artery. Eight cases out of ten presented symptoms indicating varying degrees of left ventricular failure. Apical systolic murmur was heard in all except one, who had only triple rhythm. The electrocardiograms revealed left ventricular hypertrophy in 8 cases and biventricular hypertrophy in 2 cases. Roentgenographically left atrial enlargement was observed in 4 cases. Left arterial and ventricular dimension were increased in 7 cases by echocardiography. In 5 cases of them, pulmonary artery wedge pressure or left atrial pressure was increased. One patient died in congestive heart failure at the age of 10 months and one patient died postoperatively. Other 4 patients have been managed with medication without difficulty.
Angiography ; Atrial Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Coronary Vessels ; Diagnosis ; Echocardiography ; Electrocardiography ; Heart Defects, Congenital ; Heart Failure ; Hemodynamics ; Humans ; Hypertrophy ; Hypertrophy, Left Ventricular ; Incidence ; Mitral Valve Insufficiency* ; Pulmonary Artery ; Pulmonary Wedge Pressure ; Systolic Murmurs

BACKGROUND: Bupivacaine is a potent, and commonly used, long acting local anesthetic. If accidentally injected into the systemic circulation, bupivacaine can cause lethal dysrhythmias and circulatory collapse. Attempts to treat bupivacaine induced cardiac toxicity have been varied and controversial, and they have not been very successful. The aim of this study was to investigate the electrophysiologic effects of bupivacaine in Purkinje fibers. METHODS: Effects of bupivacaine on the membrane potential were studied in 12 isolated canine Purkinje fibers. Purkinje fibers from ventricle were dissected and mounted in a tissue chamber perfused with Tyrode's solution. Transmembrane potentials recorded through glass microelectrodes filled with 3M KCI in the beating or quiescent Purkinje fibers during infusions of bupivacaine at concentratons of 3*10/-7M,10/-6M, 3*10/-6M,10/-5M, and 3*10/-5M. RESULTS: Bupivacaine reduced action potential druation in a dose-dependent manner. Bupivacaine produced a decrease in intracelullar sodium ion activity in driven(1Hz) and quiescent canine Purkinje fibers. Bupivacaine-induced hyperpolarizaton of diastolic membrane potential in quiescent Purkinje fibers was dose dependent, and the hyperpolarization by bupivacaine was attenuated by depolarization induced by high potassium extracellular concentration in part. CONCLUSIONS: These results suggest that bupivacaine decreases the fast inward sodium current, and inhibits pacemaker current in canine Purkinje fibers.
Action Potentials ; Bupivacaine* ; Glass ; Membrane Potentials* ; Membranes* ; Microelectrodes ; Potassium ; Purkinje Fibers ; Shock ; Sodium ; Sodium Channels

BACKGROUND: The effects of a newly synthesized potassium channel opener, KR-30816((-)(nitro-2-hydroxymethyl-2-methy-2H-1-benzopyran-4-y1)pyridine oxide) on the action potential of papillary muscles of guinea pigs and the ATP-sensitive potassium channel current(IKATP) of single ventricular muscle cells of rats were examined to make clear its action mechanism of the KATPchannel. METHODS: We used the conventional microelectrode and the excised inside-out patch configuration. RESULTS: KR-30816 caused a shortening of the action potential duration in dose-dependent manner, which was inhibited by glibenclamide(3microM). Before run-down of the K+channel, KR-30816 activated the cardiac ATP-sensitive K+ channel only in the presence of ATP and shifted the dose-response relation curve between [ATP]i and the channel activity to the right in parallel. After run-down of the KATP channel, KR-30816 did not after the channel opening either in the absence or in the presence of UDP. CONCLUSION: These results suggest that KR-30816 antagonizes the inhibitory effect of ATP on the KATPchannel in a competitive manner, thereby enhancing the channel openings.
Action Potentials ; Adenosine Triphosphate ; Animals ; Guinea Pigs ; Heart ; Microelectrodes ; Muscle Cells ; Papillary Muscles ; Potassium Channels* ; Potassium* ; Rats ; Uridine Diphosphate