Successful treatment of a non-haemophilic patient with inhibitor to factor VIII by double-filtration plasmapheresis.

Factor VIII (FVIII) inhibitors appear in 3-20% of haemophilia A patients after injection of FVIII concentrates. However, autoantibodies to FVIII are also reported in non-haemophiliacs. In these patients FVIII inhibitor disappears spontaneously or diminishes in response to immunosuppressive therapy. However, a few patients show resistance to immunosuppressive therapy. We describe a non-haemophilic elderly patient with acquired FVIII inhibitor who failed to respond to prednisolone. He was treated with double-filtration plasmapheresis (DFPP) which resulted in a very rapid reduction in inhibitor levels and resolution of symptoms.

[A case of successful tissue plasminogen activator in young-onset pulmonary embolism].

A 16-year-old boy was admitted to the hospital because of chest pain, dyspnea, and syncope. Physical examination revealed blood pressure of 100/60 mmHg, regular pulse of 120 beats/min, and respiratory rate of 30/min. Pulsation of the right ventricle was palpable in the left margin of the parasternum. An increased second sound was audible in the second inter-costal lesion of the left subclavicle mid-line. Results of blood tests were close to normal limits, except for slight leukocytosis and elevation of the LDH value. Analysis of artery blood gas showed hypoxia. The chest x-ray film showed cardiac enlargement. The value of systolic pulmonary artery pressure was estimated to be 47 mmHg by the cardiac echogram, which revealed enlargement of the right ventricle. Pulmonary embolism was suspected from the above findings. The value of pulmonary artery pressure was found to be 49/19 mmHg by Swan-Ganz catheter. Angiography of the pulmonary artery revealed filling defects of right in the right pulmonary artery. Tissue plasminogen activator was injected directly to the right pulmonary artery. After that, chest pain and dyspnea were relieved. In addition, arterial oxygen improved and pulmonary artery pressure decreased. At the 6th day after admission, the defect in the pulmonary artery angiography disappeared. Deep vein thrombosis of both femoral veins was recognized as a cause of pulmonary embolism by angiography of the femoral vein.

Ultrasound study in the diagnosis of primary Budd-Chiari syndrome (obstruction of the inferior vena cava).

Primary Budd-Chiari syndrome, obstruction of the hepatic portion of the inferior vena cava (IVC), is a rare disorder, but relatively prevalent in the Far East, northern India and Africa. Ultrasound examination was carried out on 9 patients with primary Budd-Chiari syndrome. There were 5 men and 4 women aged 27-60 years. In all the 9 cases, the diagnosis was confirmed by cavography and liver histology. Moreover, 7 of the 9 subsequently underwent radical operation using a patch graft. Ultrasonic study showed several characteristic findings suggestive of the syndrome, and frequencies of the main findings were as follows: 1) an echogenic obstructing membrane; 22.2%, 2) segmental obstruction of the IVC; 77.8%, 3) occlusion of the major hepatic veins at the juxtacaval portion; 100%, 4) enlarged inferior right hepatic veins; 55.6%, 5) abnormal intrahepatic venous structures and collaterals; 88.9%. Of these findings, 5) was the most prominent and most characteristic in the diagnosis of the syndrome. It is necessary for early detection of this entity to evaluate carefully intrahepatic venous abnormalities and patency of either the IVC or major hepatic veins on ultrasonic examination. The careful examination for Budd-Chiari syndrome should be done in cases with cryptogenic liver cirrhosis, particularly in countries where the prevalence of the syndrome is high.

Natural seroconversion from hepatitis Be antigen to antibody among hepatitis B virus carriers in Okinawa Islands.

In the Okinawa Islands, the great majority of hepatitis B surface antigen (HBsAg) carriers have already acquired antibody to hepatitis Be antigen (anti-HBe) by the age of 30 years (preliminary cross-sectional data). To elucidate natural seroconversion from hepatitis Be antigen (HBeAg) to anti-HBe among HBsAg carriers found in the islands of Okinawa Prefecture, 34 HBeAg-positive HBsAg carriers were followed for 1-6 years with serial measurements of aminotransferase levels, HBeAg, and anti-HBe. The 34 subjects included 24 patients with chronic hepatitis (group 1) and ten asymptomatic HBsAg carriers (group 2). During the follow-up period, HBeAg disappeared from 14 subjects in group 1 with the cumulative clearance rate of HBeAg of 56.3% within the first 2 years and with 10 of the 14 subsequently developing anti-HBe. Moreover, the aminotransferases in 12 of the 14 spontaneously seroconverted fell into the normal range. The annual clearance rates of HBeAg among group 1 and group 2 were 25.6% and 9.3%, respectively. The tendency for early disappearance of HBeAg during a carrier's life time or in the course of chronic hepatitis may lead to the low death rate from hepatocellular carcinoma (HCC) particularly HCC associated with hepatitis B virus infection in this area.

[New trial with thiabendazole for treatment of human strongyloidiasis].

Okinawa prefecture is well known as an endemic area of Strongyloides stercoralis infection, and its recent infection rate was reported 6.2%, which was investigated by a new technique to detect S. stercoralis, agar plate method. Traditional treatment with thiabendazole was temporarily effective for S. stercoralis, but the recurrence rate was extremely high. We tried the new treatment for the purpose of complete eradication of the parasite. The patients were divided into two groups, who were given 500 mg of thiabendazole three times daily for 5 days and not medicated for the following 9 days. The medication was repeated 3 times in group 1 which consisted of 92 patients and 4 times in group 2 which consisted of 70 patients. Obtained results were as follows: 1) Six months after treatment, the cure rate was 89.5% in the only one course treatment, and 100% in more than 2 course treatments. 2) Side effects such as nausea, vomiting, anorexia or general fatigue were noted in 67.5% of all the patients after initial treatment, and 45.1% of the patients were dropped out of this trial. The dose of the drug was reduced in 32.1% of the patients, and only 22.8% were treated with full course of the regimen. 3) The elevation of S-GPT was observed in 33.8% of all patients. After initial treatment the rate was only 8.1%, but after 3 or 4 repeated course of treatments the rate was elevated to 39.0% and 45.4%, respectively. The liver injury was closely related to the total dose of thiabendazole and the period of the medication.(ABSTRACT TRUNCATED AT 250 WORDS)

[Treatment of strongyloidiasis with mebendazole and its combination with thiabendazole].

Although Strongyloides stercoralis (S. stercoralis) infection rate in Okinawa Prefecture was less than 2% by the traditional method, it has been proven to be 6.2% by the new technique--agar plate method. Thiabendazole has strong activity to eradicate the organism, but it is well known that the rate of severe side effects is extremely high. Therefore, we attempted to evaluate the new treatment for the infection by mebendazole and its combination with thiabendazole. The reason for use of the drug is based on the reports of successful treatment of S. stercoralis infection in humans with the mild and infrequent side effects produced by the drug. Thirty three patients were orally given mebendazole 100 mg twice daily for 28 days. Twenty six patients were given thiabendazole 500 mg thrice daily for 5 days and after that, mebendazole 100 mg twice daily for 9 days. This combination therapy was repeated twice. The following results were obtained: 1) Out of a total of 59 patients, the cure rate was 83.3% (20/24) in single use of mebendazole and 100.0% (22/22) in the combination therapy. 2) Constipation (9.1%) and headache (9.1%) were of relatively high incidence in the mebendazole group, but they were mild. Nausea (19.2%) and headache (15.4%) were observed in the combination therapy group and the drug was discontinued in 2 patients. 3) The incidence of the elevation of S-GOT, S-GPT was noted in 71.4% (20/28) for the mebendazole group and 52.2% (12/23) for combination therapy group. All 13 patients of the mebendazole group were negative in lymphocyte stimulation test for mebendazole.(ABSTRACT TRUNCATED AT 250 WORDS)

[The epidemiological study on hepatitis B virus infection in Miyako district--relationship to the mass survey of filariasis].

It has been well known that the transmission of HB virus is associated with mass inoculation and other medical procedures. In order to make clear the relation between HBV infection and the mass survey for filariasis on which sera of the examinees were drawn from their earlobes for the detecting of microfilaria with inadequately sterilized instruments, a epidemiological study was conducted in Miyako district. Okinawa prefecture, Japan, in which both HBV infection and filariasis were prevalent. A total of 2,231 inhabitants were investigated for HBV seromarkers. The over all positive rate of HBs antigen was 6.3% with the highest rate of 15.5% in the group of age 25 to 29. On the other hand, the prevalence rate of HBs antibody continued to increase along with age group, and remained to be over 60% after 30 years of age. The positive rates of both HBs antigen and antibody in the subject district were higher than those in other place of Okinawa prefecture or Japan. Comparing yearly attack rates of HBV chronologically, which were calculated from the age-specific HBV exposure rates in the district, the annual incidence of HBV infection was 6.1% (%/year) between 1954 to 1958, 5.2% (%/year) between 1959 to 1963, and then 4.4 (%/year) in the period of 1964 to 1968 in which the proportion of examinees for the survey of filariasis was the highest. In conclusion, the mass survey for filariasis was not the main factor contributing to the high prevalence of HBV infection in Miyako district.

[A case of multiple jejunal diverticula with strongyloidiasis and malabsorption syndrome].

We reported multiple jejunal diverticula with strongyloidiasis and malabsorption syndrome. To our knowledge, 140 cases including the present one have been reported in Japan. We reviewed these cases in this report.