Alpha-Fetoprotein Subtractions in Germ Cell Tumors Identified by Con A or LCH Crossed-Line Affinity Immunoelectrophoresis

ABSTRACT: Using concanavalin A (Con A) crossed-line affinity immunoelectrophoresis and lentil lectin (LCH) crossed-line affinity immunoelectrophoresis, alpha-fetoprotein (AFP) subfractions were studied in sera including three sera from nude mice heterotran-splanted with human yolk sac tumor of the ovary and three sera from patients with yolk sac tumor, mature solid teratoma, or immature solid teratoma of the ovary. In sera of nude mice bearing yolk sac tumor or from a patient with yolk sac tumor, subfractions from yolk sac and those from fetal liver were identified. Since AFP subfractions from yolk sac and fetal liver can be differentiated according to the carbohydrate moieties, our findings indicate that AFP produced by yolk sac tumor and fetal yolk sac are to some extent differently glycosylated. We also found that AFP in both mature and immature solid teratoma was composed of two subfractions ontogenetically originating from yolk sac or fetal liver. All these findings indicate that more than two different factors are responsible for the AFP synthesis in germ cell tumor of the ovary.     

An interplay of alleviating mutations in the clinical phenotype of β‐thalassaemia intermedia

Prediction of a β‐thalassaemia major phenotype from the β‐genotype is generally relatively straightforward. However, despite the ability to accurately define the β‐thalassaemia mutations, prediction of a β‐thalassaemia intermedia phenotype from the genotype sometimes remains problematic and this has important implications in genetic counselling and prenatal diagnosis. We report a 11‐year‐old Indian male child with a thalassaemia intermedia phenotype. β‐Globin gene analysis of the family showed that he was a compound heterozygote with the ?88 (C→T) β⁺‐mutation and the IVS1 nt 130 (G→C) β⁰‐mutation. Both these mutations are rare among Indians. The propositus was also found to be heterozygous for the XmnI polymorphism and had a normal α‐genotype. In this family interplay of two alleviating mutations (a milder promoter mutation along with a gene for raised HbF) might have synergistically compensated for lack of globin chains in the patient. Hence, the nature of the β‐genotype as well as the knowledge of the presence or absence of alleviating factors will help the clinician to decide whether early commencement of a regular transfusion regime is necessary.     

Malignant Mesenchymoma of the Liver with Hypercalcemia Report of a Case

A case of malignant mesenchymoma of the liver is reported. Thediagnosis was confirmed on the surgical specimen. The patientshowed marked hypercalcemia. Parathyroid hormone assay was negative.     

A 56 Year-Old Female with Congenital Hepatic Fibrosis Diagnosed by Laparoscopy

Abstract: We describe a 56-year-old woman with congenital hepatic fibrosis. Blood tests and liver scanning with Tc-99m-labelled galactosyl human serum albumin revealed mild liver dysfunction. Per-rectal portal scintigraphy with iodine-123 iodoamphetamine showed severe abnormalities in the portal circulation, and the portal pressure measured during percutaneous transhepatic portography was high (350 mmH₂O). Idiopathic portal hypertension was suspected. Laparoscopy disclosed diffuse, intense dendritic white markings around the liver. Congenital hepatic fibrosis was confirmed on histologic examination of a biopsy specimen obtained during laparoscopy. In summary, we report a rare and relatively elderly case of CHF, in which laparoscopy was useful in the diagnosis. (Dig Endosc 1999; 11: 174–178)     

Peritoneoscopic Findings of Autoimmune Hepatitis

Abstract: To clarify the morphologic differences between hepatitis C virus (HCVI-negative autoimmune hepatitis (AIH) and HCV-positive AIH, peritoneoscopic findings were studied. Among twenty three patients with AIH according to the Japanese criteria (1992), 15 were HCV-negative and 8 were HCV-positive. The terms grooved depression, coarse depression, coarse elevation, coarse undulation, and round-shaped reddish marking (RM) were used in this study to evaluate the peritoneoscopic findings. Grooved depressions, coarse depressions, coarse elevations, coarse undulations and round-shaped RMs were all common findings (53%, 87%, 73%, 80%, and 80%, respectively) in HCV-negative AIH patients, but they were less common (13%, 25%, 13%, 13%, and 0%, respectively) in HCV-positive AIH patients. This study revealed that HCV-negative AIH patients had different peritoneoscopic findings from HCV-positive AIH patients. Thus HCV-negative AIH may be typical AIH, and HCV-positive AIH may essentially be a subset of type C chronic hepatitis.     

Hepatic HCV-RNA as a predictor of outcome after interferon therapy in patients with chronic hepatitis C

Measurement of serum HCV-RNA is a useful index for evaluating the antiviral effect of interferon therapy in chronic hepatitis C. In the present study, we investigated whether the detection of hepatic HCV-RNA after interferon treatment, using a polymerase chain reaction assay, predicted long-term response to therapy in patients with chronic hepatitis C. Thirty-three patients underwent liver biopsies before and after interferon therapy. Histology and clinical courses were compared after treatment. Before therapy, serum and hepatic HCV-RNA was detected in specimens from 32 (97%) and 33 (100%) patients, respectively. Serum HCV-RNA became undetectable in samples from 22 (67%) patients; however, in 10 of these patients (45%), serum HCV-RNA levels relapsed after therapy. Hepatic HCV-RNA became undetectable in 14 patients after therapy and the serum aminotransferase concentration remained within normal limits during and following (24–92 weeks) therapy in 12 of these patients (86%). All 11 patients with detectable hepatic HCV-RNA also had serum HCV-RNA and elevated aminotransferase concentrations refractory to therapy. The absence of hepatic HCV-RNA at the end of interferon treatment thus predicted a long-term complete response to therapy with a sensitivity of 100%, a specificity of 90% and an accuracy of 94%. We conclude that hepatic rather than serum HCV-RNA is a more useful index for the prediction of the long-term efficacy of interferon therapy.     

Efficacy of Prophylactic Endoscopic Variceal Sclerotherapy in Patients with Hepatocellular Carcinoma

Abstract: A setrospective study to determine the influence of prophylactic endoscopic sclerotherapy on the survival rate of patients with hepatocellular carcinoma complicated by esophageal varices was conducted. The subjects included 132 patients without esophageal varices at the time of diagnosis (NV group), 37 patients with hepatocellular carcinoma for whom prophylactic sclerotherapy had been performed for risky esophageal varices (PS group) and 26 patients with hepatocellular carcinoma and risky varices for whom prophylaxis was not performed (NPS group). Multiple regression analysis was used to identify factors affecting the survival rate of the 132 patients without esophageal varices. A tumor embolus in the primary branch or main trunk of the portal vein adversely affected long-term survival in these patients. The patients were further divided into 2 subgroups, namely those with (Vp3) and without (Vp0-Vp2) tumor emboli in the main trunk and primary branch of the portal vein. In the Vp0– Vp2 emboli subgroup, the patients who received prophylactic sclerotherapy and the patients without varices had similar survival rates. Patients without prophylactic sclerotherapy had a significantly shorter survival rate than the group without varices and the prophylactic sclesotherapy group (p<0.01 and p<0.05). A significant bleeding rate was observed in the group without prophylaxis and not in the group with prophylaxis. However, in the Vp3 subgroup, the survival rate in each group was identical. Thus prophylactic variceal sclerotherapy is indicated for patients with hepatocellular carcinoma who do not have tumor emboli in the main trunk or primary branch of portal vein.     

Anti-neutrophil cytoplasmic antibody for proteinase 3 in a child with polyarteritis nodosa

We report a case of a 15 year old boy with polyarteritis nodosa associated with antineutrophil cytoplasmic antibody (ANCA) against proteinase 3 (PR3). After months of steroid and immunosuppressant therapy, the symptoms subsided and the polyaneurysms almost disappeared. The levels of anti-PR3 antibody and of cytokines also decreased. The results indicate that ANCA is a good indicator of this disease activity, and may play some pathogenic role in the disease.