Fatal hepatic hemorrhage by peliosis hepatis in X-linked myotubular myopathy: a case report.

We report a 5-year-old boy with X-linked myotubular myopathy complicated by peliosis hepatis. At birth, he was affected with marked generalized muscle hypotonia and weakness, which required permanent ventilatory support, and was bedridden for life. He died of acute fatal hepatic hemorrhage after using a mechanical in-exsufflator. Peliosis hepatis, defined as multiple, variable-sized, cystic blood-filled spaces through the liver parenchyma, was confirmed by autopsy. To avoid fatal hepatic hemorrhage by peliosis hepatis, routine hepatic function tests and abdominal imaging tests should be performed for patients with X-linked myotubular myopathy, especially at the time of using artificial respiration.

[A case of Legionella pneumonia successfully treated intravenously with both erythromycin and ciprofloxacin].

A 54-year-old man was admitted to our hospital complaining of a high fever and watery diarrhea. The chest radiograph on admission revealed a homogeneous consolidation of the left upper lobe. Laboratory findings included proteinuria, oligouria, hematuria, myoglobinuria, hyponatremia, and serum CPK elevation. On the basis of these findings, a tentative diagnosis of Legionella pneumonia was made. He was treated with sulbactam/cefoperazon and erythromycin, but his high fever remained and the consolidation shadow deteriorated. He was therefore given both erythromycin and ciprofloxacin intravenously. After several days the fever had returned to normal, the appearance of the chest radiograph had improved, and his symptoms were quickly relieved. This case suggests that intravenous administration of ciprofloxacin and erythromycin can be an effective treatment against Legionella pneumonia.

[Home oxygen therapy (HOT) in patients with pulmonary tuberculosis sequelae--comparison between patients medically treated and those surgically treated].

In Japan there are about 40,000 patients under home oxygen therapy (HOT), of whom about 30 to 40% are pulmonary tuberculosis sequelae (TBS). These patients can be divided into three groups depending on the treatments they had, Group 1: those who had medical treatments only, Group 2: those who had artificial pneumothorax, and Group 3: those who had thoracoplasties or other surgical treatments. The purpose of this study was to observe the distributions and possible differences in the survival rates among these groups. The study included 1537 patients with TBS under HOT followed at National Hospitals and Sanatoriums nationwide in Japan. In 819 patients the treatments were specified and of those 354 were in Group 1, 29 in Group 2, and 436 in Group 3, so that the proportion of surgically treated patients in PTS was estimated between 28.4% (436/ 1537) to 53.2% (436/819). The ages at the onset of tuberculosis, at the start of HOT and the intervals in between were 36.6, 66.2 and 29.8 in Group 1, and 26.8, 65.5, and 38.1 in Group 3 respectively. Though the ages at the start of HOT were the same, those at the onset of tuberculosis were about ten years younger in Group 3 than in Group 1. Comparing Group 1 and 3, the survival rates after the initiation of HOT (Kaplan-Meier method) was better in Group 2 (surgically treated) than in Group 1 (medically treated). It is speculated that the reason could be a better preservation of the function of the remaining lung in the surgically treated and a higher incidence of obstructive impairments in the medically treated patients.

[Studies on the nontuberculous lung mycobacteriosis in Japan. (Report of the study in the year 1987 and 1988 of the Mycobacteriosis Research Group of the Japanese National Chest Hospitals)--pulmonary infection caused by Mycobacterium kansasii has begun to appear in all over Japan including north Japan Hokkaido].

UNLABELLED: In this study, the Mycobacteriosis Research Group of the Japanese National Chest Hospitals (MRG) presents the reports of study years 1987 and 1988. As reported previously**, pulmonary infection caused by Mycobacterium kansasii occurred principally in South-West Japan (prefectures South-West of Tokyo) and did not appear in North Japan. However, this disease appeared in 1987 and 1988 in Hokkaido (Sapporo Hospital). Accordingly, we may say the disease occurs all over Japan. This is a noteworthy finding newly recognized in the study years. The prevalence rate of nontuberculous lung mycobacteriosis was determined as 2.92 or 2.78 in 1987 and as 2.02 or 1.91 in 1988 per 100,000 population per year. The estimated rates based on the ratio of nontuberculous lung mycobacteriosis against active lung tuberculosis and based on the ratio of nontuberculous lung mycobacteriosis against culture-positive lung tuberculosis well agreed with each other. COMMENT: In this country, chest physicians customarily report their cases of nontuberculous mycobacteriosis including lung tuberculosis, because the payment of treatment for patients with tuberculosis is free. Because of this custom, tuberculosis statistics surely contain cases of nontuberculous mycobacteriosis. Caution about this has been paid in calculating the prevalence rate. From the study year 1987, the MRG chairman moved from Michio Tsukamura, The National Chubu Hospital, to Nobuhiko Kita, The National Kinki Chuo Hospital.

Dye penetration of the smear layer and fluoride application to the dentin surface.

A smear layer is formed after cavity or root canal preparation. The aim of the present study was to reinforce the dental surface in order to prevent the invasion of foreign irritants, by treating the smear layer with fluoride. Dentinal samples whose surfaces had been washed with water after the formation of a smear layer, and corresponding samples without washing, were examined by the dye penetration test, and the results were compared. Although there was no significant difference between the two groups of samples, dye penetration was suppressed by about 30% in washed samples, whereas the suppression was 20% in unwashed samples. When washed samples were treated with 1.0% SnF2, 10.0% SnF2, 7.5% Na2PO2F, 15% Na2PO2F, APF, 10 mM In(NO3)3, 100 mM TiF3, 50 mM TiF3, or 10 mM TiF3, samples washed after treatment with 1.0% SnF2 showed a dye penetration suppression of about 60% as a whole, in comparison with samples having no smear layer. Hardly any suppression of dye penetration was observed after treatment with other fluorides.

Situs ambiguous with polysplenia complicated by renal adenocarcinoma.

Two men developed renal adenocarcinoma in association with situs ambiguous off with polysplenia (SAP) (also known as the polysplenia syndrome). Features of their diseases included (1) no normal spleen--just splenuli, (2) interruption of the inferior vena cava with azygos or hemiazygos continuation, (3) bilateral hyparterial bronchi, (4) cardiac malformations, (5) renal adenocarcinomas originating from the kidneys, ipsilateral to the anomalous spleens. The association of renal adenocarcinomas and SAP has not been previously reported, to our knowledge. We suggest that renal adenocarcinoma and SAP may share a common pathogenetic link.

Galactosyltransferase activities in human sera of various diseases.

Recently galactosyltransferase has been noted as a new tumor marker. In this report galactosyltransferase activity was measured in sera of various diseases including malignant tumors and the levels of the enzyme activities were compared. The levels of the enzyme were higher in sera of malignant tumors and liver diseases than those in sera of other diseases. In malignant tumors little organ specificity was found with respect to the levels of galactosyltransferase activities. The levels of the enzyme were considered to have no relation to carcinoembryonic antigen or alpha-feto protein. On the other hand, in liver diseases the levels of the enzyme were correlated to the severity of liver damage. Therefore, it is reasonable to presume that the elevation of serum galactosyltransferase activity is caused chiefly by malignant tumors and liver damage.

A case of meglumine iodipamide hepatotoxicity.

There have been only two reports of severe hepatotoxic reaction caused by meglumine iodipamide. Lately we experienced such a reaction in an 66-year old female with chronic intrahepatic cholestasis. After drip infusion cholangiography was performed by infusing 40 ml. of 50% meglumine iodipamide (Biligrafin) intravenously, the patient developed nausea and abdominal pain. Her serum transaminase rose to more than 2,000 K-A units on the third day and gradually returned to normal by the 18th day. The macrophage migration inhibition test of her blood was positive for meglumine iodipamide. Accordingly some delayed type of hypersensitivity in the above reaction could be considered. When a larger amount than a recommended dose of meglumine iodipamide is infused in cholangiography, a severe hepatotoxic reaction might be induced, especially in icteric cases.

Hepatic bruits in malignant liver tumors.

There have been few reports concerning hepatic bruits in malignant liver tumors, especially in those other than hepatocellular carcinoma. Lately we noticed hepatic bruits in several cases of malignant liver tumors. The murmurs were confirmed by sound recordings. In cases of hepatocellular carcinoma the murmurs were continuous or continuous with late systolic accentuation. In a case of cholangiocarcinoma of the liver the murmur was continuous with late systolic accentuation and in a case of metastatic liver carcinoma it was systolic. These murmurs were considered to be caused by various mechanisms and some of them are discussed in this paper. Hepatic bruits would be a useful aid in diagnosing hepatic tumors.

Low voltage optical modulator using electrooptically induced phase gratings.

An optical modulator with square phase gratings, which are induced by a mosaic structure of electrooptic crystals, is proposed. Analysis and experiments show its utility and the possibility of fabricating it by binding several crystals. An experimental device is built by an array structure using eight 0.9(z) x 0.9(y) x 12.5(x)-mm LiNbO(3) rods. Its 100% modulation voltage is 68 V, and its capacitance is 18 pF. Experimental results, such as modulation transfer function and angle of diffraction, agree well with the analysis, and the farfield patterns obtained are fairly good. This type of modulator offers a low voltage operation, which is about 40% of that of a conventional one for LiNbO(3)crystals. Furthermore, it has the advantage of insensitivity to temperature change.