Pseudoaneurysm after Renal Biopsy; Angigraphic Diagnosis and Treatment by Superselective Embolization

The use of superselective embolization was assessed as a treatment for bleeding from arteriovenous fistulas and pseudoaneurysm after renal biopsy procedure. But unless it is sufficiently selective, the procedure results in loss of significant amount of renal parenchyme. We experienced one case of renal arterial pseudoaneurysm, which happened at 5days after percutaneous renal biopsy. Diagnosis of pseudoaneurysm was made by ultrasound examination with Doppler flow and was confirmed with arteriography. The rupture of pseudoaneurysm was occluded by transcatheter embolization with placement of a steel coli and polyvinlyl alcohol from the renal arterial approach. This procedure was allowed non surgical closure of the AV fostula and pseudoaneurysm without significant change of renal function.

A Case of Idiopathic Long QT Syndrome with 2:1 Atrioventricular Block

No abstract available.

Clinical Analysis of Serum and Urine N-Acetyl-B-D-Glucosaminidase(NAG) in Renal Disease

Recently, NAG activity has gained increasing importance as and aid in the diagnosis of renoparenchymal diseases. Elevation of urine NAG activity has been found to be an indicator of renoparenchymnal diseases. To evaluate the diagnostic value of the NAG activity test in the renal disease, we carried out clinical study on 31 cases of renal disease patients who had been admitted to the Department of Pediatrics, Chung-Ang University Hospital between March 1992 and February 1993. We analyese by two data: (1) Stastical Package for the Social Science. (2) Students'T test. The results were as follows 1) The urine NAG activity significantly increased (p<0.05) to 69.9+/-5165.69U/hr/mg Creatinine (U/hr/mg Cr) in the renal disease group compared to 3.6+/-1.91U/hr/mg Cr in the control group. 2) The serum NAG activity was 11.69+/-5.18U/L in the renal disease group and 10.58+/-4.04U/L in the control group. There was no significant difference in the serum NAG sctivity between two groups. 3) In the renal disease group, the serum NAG activity was 10.78+/-3.32U/L in male 12.53+/-6.47U/L in female. There was no significant difference between both sexes. In the renal disease, the urine NAG activity was 33.62+/-30.67U/hr/mg Cr in male and 114.05+/-241.62U/hr/mg Cr female. There was no significant difference between both sexes (p<0.05). 4) In the renal disease group, the urine NAG activity increased 314.73+/-420.39U/hr/mg Cr in the 2 years old group but there was no significant difference of urine NAG activity compared to above 2 years old group . 5) The urine NAG activities were 93.1+/-0193.04U/hr/mg Cr in the poteinuria subgroup and 13.3+/-47.62U/hr/mg Cr in the nonproteinuria subgroup and 3.66+/-1.91U/hr/mg Cr in the control group. There was no significant difference between the proteinuria subgroup and the nonproteinuria subgroup. but there was significant difference between nonproteinuria subgroup and control grouop(p<0.005).

A case of Roberts syndrome

Roberts syndrome is an autosomal recessive disorder accompanied by limb defects, craniofacial abnormalities, pre-and postnatal growth retardation. Patients with Roberts syndrome have characteristic premature separation of heterochromatin of many chromosomes and abnormalties in celldivision cycle. We have experienced a case of Roberts syndrome in an immature neonate The patients showed characteristic clinical features of multiple, severe facial mid-line clefts, and tetraphoco-amelia. The brief review of the literlature was made.

A case of Fanconi syndrome

Fanconi syndrome is a complex of renal tubular dysfunction defined by glycosuria without diabetes, generalized aminoaciduria, phosphaturia, bicarbonaturia, uric aciduria, and renal tubular acidosis. It is often associated with hypokalemia, hypophosphatemia, rickets and osteomalacia. We have experienced one case of Fanconi syndrome with chronic tubulointerstitial nephritis. The patient was a 4 year old and his chief complaints were polyuria, polydipsia, and poor weight gain. There were hyperchloremic metabolic acidosis, hypokalemia, glycosuria, generalized aminoaciduria and phosphaturia. We report a case of Fanconi syndrome with brief review of the literatures.

A case of malignant atrophic papulosis (Degos' disease)

No abstract available.

Echocardiographic Evaluation of Sequential Change of Cardiac Function in Normal Neonates

No abstract available.

A Case of Congenital Hypofibrinogenemia

No abstract available.

Clinical Study on Total Anomalous Pulmonary Venous Return

Clinical study was performed on 42 patients diagnosed as TARVR at the Departmet of pediatrics, Seoul National University Hospital, from January, 1966 to June, 1982. The results are as follows, 1) Of the 25 cases with isolated TAPVR, age distribution was from 10 days to 10 years. Seventeen cases were male and eight cases were female. Of the 24 cases with complicated TAPVR, age distribution was from 2 days to 22 years. Eighteen cases were male and six cases were female. 2) The type of TAPVR was supracardiac in 29 cases (59.1%), cardiac in 10 cases (24.4%), infradiaphragmatic in 3 cases (6.1%) and mixed in 5 cases (10.2%). 3) Birth weights of patients were within normal limits except 1 case. 4) The most freuent complaints at diagnosis were tachypnea and cyanosis. 5) PDA was the most frequently associated intracariac anomaly (54.1%) which was followed by pulmonary stenosis (16.6%), single atrium (16.6%) and single ventricle (16.6%). 6) Of the 25 cases with isolated TAPVR, chest roentgenographic findings were cardiomegaly in 22 cases (88%) and increased pulmonary vascularity in 23 cases (92%). 7) Of the 25 cases with isolated TAPVR, the ECG showed right axis deviation in 22 cases (88%), right atrial enlargement in 13 cases(52%), right ventricular hypertrophy in 24 cases (96%) and combined ventricular hypertrophy in 1 cases (4%). 8) Of the 23 cases with isolated TAPVR, echocardiographic finding showed increased dimension of the right ventricle in all cases. Of the 39 cases with TAPVR, the common venous chamber was visualized posterior to the left atrium in 14 cases(35.8%). 9) Cardiac catheterization was performed in 24 cases with isolated TAPVR. Pulmonary hypertension was observed in 19 cases (79.1%). 10) Fourteen patients with isolated TAPVR were operated. Three patients died.