Biomedical subjects
Publications and source records attributed to M H Chang.
Rotor's syndrome is a rare hereditary non-hemolytic hyperbilirubinemia. This report concerns an 11-year-old boy who presented with fluctuant jaundice since birth. Liver function tests showed conjugated hyperbilirubinemia with normal serum aminotransferase and globulin level. The diagnosis was confirmed by laboratory workup which included normal liver histology and oral cholecystography and typical sulfobromophthalein excretion curve.
Fifty-five patients with cytomegalovirus (CMV)-associated neonatal hepatitis (NH) were followed for 12 to 90 months. Six patients (10.9%) died from either a fulminant course or a chronic liver disease. Among the remaining 49 patients, whose liver function was completely recovered, there were eight with retardation of developmental or growth status, and two with hearing impairment. Overall, 20.4% of the survivors suffered from a long-term impact. The unfavorable outcome was related to several clinical and pathological parameters. These included persistence of clay-colored stool, presence of splenomegaly, ascites or anemia, high peak total and direct bilirubin, low nadir albumin levels, diffuse giant cell transformation and cirrhosis of the liver. The seropositivity of CMV infection did not significantly correlate with the outcome.
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.
One hundred and four cases of Hirschsprung's disease in infants and children diagnosed at the National Taiwan University Hospital during the period of 1984 to 1989 were analysed for demographic patterns, clinical manifestations, accuracy of various diagnostic procedures, especially the reliability of suction biopsy, and postoperative course. The important symptom constellation included abdominal distention, delayed meconium passage, and tight anus in the biopsy-positive group. Complementary diagnostic procedures were mandatory for accurate diagnosis, since 26% of the patients (7 of them with ultrashort segment) were diagnosed over a period of more than two months. The first biopsy in 25% of the patients aged less than 1 month could not be interpreted, and in 27.6% of this subgroup the radiological pictures were inconclusive. Further scrutiny of clinical manifestations together with other elaborate study including repeated rectal biopsies may be required for establishing a definite diagnosis of the disease. The surgical mortality is low, but surgical morbidity remains high and needs further improvement.
Wilson disease presenting as fulminant hepatic failure, severe hemolysis and renal failure is rare in the literature. A ten-year-old boy--complaining of abdominal pain, jaundice, tea-colored urine, and anemia was admitted to this hospital; examination showed Kayser-Fleischer rings, anemia associated with hemolysis, mildly elevated serum transaminases, extremely elevated bilirubin levels, low serum ceruloplasmin level, slightly elevated serum copper, excessive 24-hour urine copper excretion, and severe renal function insufficiencies. Under the impression of Wilson disease with fulminant hepatic failure, the patient was treated by oral D-penicillamine 1 gm per day, intravenous zinc sulphate (about 8 mg per day elemental zinc), and given other supportive treatment. Unfortunately, the patient died of hepatic failure complicated with septic shock 21 days after the onset of symptoms. Autopsy found liver copper content was 586.92 ug/gm dry weight and kidney copper content: 300.19 ug/gm dry weight, abnormally high as compared with normal tissue. A review of the literature led to conclusion that the best treatment for Wilson fulminant hepatic failure is liver transplantation.
A 10-year-old mentally retarded girl was sent to our hospital due to generalized edema, vomiting and poor appetite for several days. Serum albumin level was low, but no proteinuria was detected. Her stool was bulky and foul. Stool examination for parasite with formalin-ether concentration method revealed negative result. Trypsin activity test of stool revealed low trypsin activity as compared with normal specimen. Daily fecal fat exceeded upper normal limit. The diagnosis of giardiasis was confirmed by duodenal juice examination. Intestinal histology revealed mild shortening of the villi with increased mononuclear cell infiltration in the lamina propria. The daily stool amount decreased markedly after treatment with metronidazole 250 mg three times a day for 7 days. The edema subsided during the treatment. Serum albumin bevel returned to normal after the treatment. Giardiasis with malabsorption syndrome has often been overlooked in Taiwan. It is advised that in case of malabsorption syndrome giardiasis should be included in the list of differential diagnosis.
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SerumIgG antibody titer against viral capsid antigen(VCA) of Epstein-Barr virus (EBV) was measured by indirect immunofluorescence antibody method in 1350 children under 15 years of age, 723 males and 627 females, who were living in the Taipei area. Sera were collected from June through October 1984. Under the age of 6 months, the prevalence rate of positive serum VCA antibody was 38.4%. It decreased to 6.4% between 6 and 12 months of age, but rose to 65.8% during the second year of life. The rate continued to increase from 1 to 4 years of age, with a steep rise of geometric mean titer in the same period. The prevalence rate stayed at more than 90% after 4 years of age. From our data, it is obvious that most children in the Taipei area had been infected by EBV in early childhood, especially from 1 to 4 years of age.
A 10-year-old mentally retarded girl from an asylum in northern Taiwan was diagnosed to have giardiasis with malabsorption syndrome at the Department of Pediatrics, National Taiwan University Hospital in 1983. A survey for giardiasis in 239 children living in the same asylum was therefore performed. Stool specimens were collected from all children, and giardia was examined simultaneously by formalin-ether concentration method and polyvinyl alcohol preservation followed by Trichrome stain. Forty one cases (17.2%) of giardiasis were detected by the former method while 48 cases (20.1%) by the latter method. Abnormally low stool trypsin activity was found in 38 of the 42 cases (90.5%) tested and the activity returned to normal in 50% of patients after successful treatment. Endoscopic examination and intestinal biopsy of upper gastrointestinal tract were performed in 12 cases. Among them, 4 were found to have nodular lymphoid hyperplasia, lymphoid hyperplasia in 7, and increased mononuclear cell infiltration in lamina propria in 7. Forty patients were treated with metronidazole 250 mg three times a day for 5 days. Follow-up stool examinations revealed that 12 children (30%) still passed giardia in their stools 4 months after treatment. Reinfection and inadequate sensitivity of the initial screening test may be used to account for such a high rate of treatment failure.
The first case, a two-month-old female infant, had frequent vomiting since 5 days of age. Dilated esophagus with narrowed distal end was noted on barium meal study. Panendoscopic examination revealed dilated esophagus and a pin-head sized, stenotic lumen at 15 cm from the incisor. Under the impression of esophageal stenosis, gastrostomy was performed for feeding. Esophagogram made at 6 months of age demonstrated good patency of the esophagus without stenosis and gastrostomy was subsequently closed at the age of 1 year. Unfortunately, swallowing disturbance had bothered her intermittently since. Barium esophagogram and panendoscopy made 8 years later again revealed distal esophageal obstruction with dilated proximal esophagus. The second case was a two and half year old boy who suffered from frequent regurgitation of undigested food for 6 months. Dilated esophagus with narrowing of the lower end of esophagus was noted on barium meal study. After admission, panendoscopic examination revealed the esophagus was very narrow at 25 cm from incisor and the proximal esophagus was dilated. The esophageal dilatation with mercury-weighted bougies was performed intermittently. At present he is doing well, and a barium meal study showed adequate esophageal emptying.
Three cases of gallbladder hydrops associated with Kawasaki disease are presented. The initial manifestations were high fever, jaundice and distended abdomen with guarding. The first two cases received laparotomy under the impression of suppurative cholecystitis with peritonitis. A markedly distended acalculous, nongangrenous gallbladder was noted. A cholecystostomy for drainage was performed. Diagnosis of Kawasaki disease was made only when the clinical manifestations became full-blown postoperatively. Both patients led an uneventful postoperative course. The third case had apparent features of Kawasaki disease at admission though the abdominal symptoms were rather prominent. With supportive care, the patient stabilized by the sixth hospital day without complication and did not require surgical intervention. We suggest that the preferred treatment of abdominal symptoms in Kawasaki disease is medical, and surgical intervention is deserved only for the complications of the hydrops. Simple cholecystostomy seems to be safe and sufficient for such occasion. Ultrasonography is helpful for the correct diagnosis of gallbladder hydrops and can exclude dilatation of the intrahepatic biliary trees and cholelithiasis.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
To study the epidemiology of Helicobacter pylori (H. pylori) infection in children, the presence of the IgG antibody against H. pylori was evaluated by enzyme-linked immunosorbent assay (ELISA) method in unselected children in Taipei city. Serum samples were collected there from 428 apparently healthy children under 12 years of age. The overall prevalence of H. pylori infection was 8.4%. A low prevalence was found in the groups 1-3 years old (0.9%) and 3-6 years old (3.7%). The prevalence rate increased significantly after 6 years of age, and reached 19.4% at 9-12 years of age. In conclusion, H. pylori infection was independent of gender, but increased with age.
A six-year-old aboriginal girl living in northeast Taiwan, was admitted via Emergency Service with the chief complaint of epigastralgia for one day. Fever, leucocytosis, hyponatremia, acidosis and unilateral pleural effusion were noted. The serum amylase was 2976 U/L. Image studies including abdominal sonography and computerized tomography revealed a swollen and edematous pancreas. There was no evidence of previous trauma, drug using, hereditary problems, nor biliary tract stone; the patient was noted to have adult worms and eggs of Ascaris lumbricoides in stool and vomitus. Ascariasis associated with pancreatitis was suspected. She recovered completely after antihelmintic therapy (mebendazole) and supportive treatment. Fourteen ascarides and 20 trichurides were expelled during nine days of admission.
Pyogenic liver abscess, which may lead to devastating consequences, is an uncommon medical problem in pediatrics and has generally been reported in compromised hosts. This article describes two patients with beta-thalassemia major and hemochromatosis complicated by Klebsiella pneumoniae liver abscess. One of the patients had severe complications, including subphrenic abscess, pleural effusion and meningitis. To present knowledge, the occurrence of K. pneumoniae liver abscess in patients with beta-thalassemia major has never before been reported in the literature.
To evaluate total parenteral nutrition-associated cholestasis (TPN-C) in infants, a retrospective clinicopathologic study was conducted of 15 infants who had received TPN. The mean gestational age and birth weight were 32.1 weeks (26-40 weeks) and 1807 g (840-5840 g) respectively. Two-thirds of the patients were kept on TPN for more than 60 days. The onset of rising direct bilirubin ranged 2-9 weeks (mean 4.5 +/- 2.4) after TPN therapy. Preterm babies less than 32 weeks of age had an earlier rise of direct bilirubin and AST. Bile sludge of the gallbladder was observed in only one case, and none had gallstone. The main histologic findings of liver biopsy or autopsy were cholestasis (intracellular and canalicular), periportal inflammation, fibrosis and bile ductular proliferation. Sixty percent of these survived, the remaining 40% died of complications unrelated to TPN-C. The liver function profile became normalized within a mean of 14.0 +/- 9.4 (8-34) weeks after discontinuation of TPN in the survival cases. It was concluded that infant TPN-associated cholestasis was mostly reversible, but that the younger preterm babies were susceptible to a prolonged TPN course with more marked clinical and pathological changes.