During the twenty-nine-year period from June 1959 to October 1988, 32 infants and children with histology-proved hepatoblastoma were seen at the Department of Pediatrics, National Taiwan University ...Hospital (NTUH). The age at diagnosis ranged from four days to six years, with a mean of one year and eight months. Most of the patients (87.5%) were under the age of three. There was no sex predilection (16 males, 16 females). The most common presenting signs were an upper abdominal mass (71.9%) and/or abdominal distention (62.5%). Physically, all the patients revealed hepatomegaly, with or without mass. Laboratory abnormalities included primarily thrombocytosis (64.3%), elevated serum aspartate aminotransferase (75.0%), serum alanine aminotransferase (62.5%), cholesterol (78.3%) and alphafetoprotein (92.3%). According to the classification of Ishak and Glunz, 56.3% of these belonged to the epithelial type; the others belonged to the mixed type. During the follow-up period, 6 patients with complete resection of the tumor remained alive from 2 years to 17 years and 4 months. Twenty-two patients died from 8 days to 16 months after diagnosis. Four patients were lost to follow-up. The two-year survival rate was 21.4% (6/28). Complete resection of the tumor was the key treatment for achieving survival.
Thirty cases of early-onset congenital diaphragmatic hernia were admitted to National Taiwan University Hospital from 1976 to 1990. The mortality rate has increased in recent years, with an overall ...mortality rate of 26.7%. Low PH value of initial blood gas, early onset of symptoms at birth and total absence of hemidiaphragm were the three most poor prognostic factors (P less than 0.01). Preoperative stabilization of those patients with an initial PH value less than 7.2 may be helpful (P less than 0.04).
A program of rapid mass screening of kidneys was conducted with ultrasound for initial detection of renal and the adjacent organ abnormalities in school children and neonates in Taipei area from ...March 1987 to December 1990. More than a half million population were examined and the frequency of the abnormality was approximately 0.5%. Of the patients 22 had a tumor, including 13 boys and 9 girls. Age ranges were from neonate to 15 years. The tumor site for 19 patients was in the retroperitoneal space, 6 cases from the kidney, 3 cases from the adrenal gland, 1 case from the pancreas, and 9 cases from retroperitoneal tissue. The other three cases had tumor in the mediastinum, ovary or bladder. In this series, 20 cases received surgery. Pathological examination revealed 8 cases to be malignant, and 12 to be benign lesions. This experience would seem to indicate that rapid renal ultrasonic mass screening may be useful for early detection and management of tumors of the kidney and its adjacent organs.
Congenital solitary kidney with hydronephrosis is a rare anomaly. Five cases including 4 boys and 1 girl were detected to have this disease in the last 3 years. Of the patients, a case had ...hydroureter. Ages ranged from 8 to 12 years. The intermittent abdominal pain was complained in one case. The enuresis was noted in another case. The other 3 cases were asymptomatic. The anomaly in these patients incidentally detected with ultrasound and then was confirmed by intravenous urogram and/or radionuclide scan. Diuretic radionuclide renogram T1/2 was used in these patients. A patient with UPJ obstruction was demonstrated and received surgery. Others were non-obstructive and followed up at OPD. Since sonography is a noninvasive modality, it can serve as a tool for long-term followup of such nonoperative patients for the evidence of urinary infection and stone formation, and the status of the functional solitary kidney.
