Ultrastructure of the hindgut of Drosophila larvae, with special reference to the domains identified by specific gene expression patterns.

The hindgut of Drosophila larvae consists of nine domains that have been distinguished by specific gene expression patterns. In the present study, we examined the ultrastructure of the hindgut of Drosophila larvae, with special reference to the domains, in order to determine whether or not the domains are morphologically distinct functional units. Each domain showed specific ultrastructural features that suggested specific corresponding functions. According to the morphological features, terms are proposed for each domain: the imaginal ring; the "pylorus," which has a thick cuticular layer and well-developed sphincter muscles; the "large intestine," which occupies a major middle portion of the hindgut and has a unique dorsal and ventral subdivision; "border cells," which delineate the anterior and posterior borders of the large intestine and the border between the dorsal and ventral domains of the large intestine; and the "rectum," which is situated at the posterior end of the hindgut and has a thick cuticular layer and sphincter muscles. The morphological features indicate that the large intestine has active absorptive activities. The domains, which have been distinguished by gene expressions, were demonstrated to be functional tissue units of the gut.

Effect of dietary antioxidants on the susceptibility to hepatic microsomal lipid peroxidation in the rat.

The inhibitory effect of probucol on ferrous-iron-induced microsomal lipid peroxidation was compared to that of alpha-tocopherol and butylated hydroxytoluene (BHT). Male Wistar rats were fed with diets containing 1% probucol, 0.2% BHT or 0.1% alpha-tocopherol for 30 days. There were no effects of dietary antioxidants on growth parameters, although liver weight was significantly higher in the BHT-fed rats. Probucol reduced serum levels of total and free cholesterol, while BHT feeding increased the concentrations of serum thiobarbituric-acid-reactive substances, HDL cholesterol and hepatic phospholipid. alpha-Tocopherol had no effect on these parameters. Incorporation of both, probucol and alpha-tocopherol, decreased the susceptibility of microsomes to lipid peroxidation in vitro, while BHT incorporation increased hepatic microsomal lipid peroxidation. These results suggest the possible usefulness of probucol for treatment of both hypercholesterolemia and elevated hepatic microsomal lipid peroxidation, while alpha-tocopherol decreases only an elevated lipid peroxidation. BHT works as a prooxidant.

Effects of dietary fat levels on the absorption and tissue accumulation of probucol in the rat.

The effects of dietary fat level on the absorption and tissue accumulation of probucol (PB, CAS 23288-49-5) were studied in male Wistar rats. Addition of 1% PB (w/w) to the diets as compared to the diets without an addition (P) resulted in a decrease in the concentration of both serum and high-density lipoprotein (HDL) cholesterol as well as TBA-reactive substances (TBARS), without influencing food intake or growth. When rats were fed with the PB diet, the apparent intestinal absorption of PB was 7.9%, while the serum PB level was 5 micrograms/ml. The tissue distribution of PB, when expressed per g of tissue, was highest in the adrenal glands, followed by the liver, heart, epididymal adipose tissues, kidneys, lung, spleen, and testes. The addition of 10% olive oil (PBO) to the PB diet (w/w) appeared to double the PB diet-induced increase in apparent intestinal absorption and the serum concentration of PB. Furthermore, compared with the PB diet, the PBO diet caused 3- to 4-fold higher accumulation of PB in the liver and its subcellular fractions and 2-fold higher accumulation in the spleen. However, there was no further accumulation of the drug in the heart, kidneys, lungs, and testes. Incorporation of the drugs into adipose tissues was significantly lower with the PBO diet than with the PB diet. These results suggest that dietary manipulation leads to changes in the intestinal absorption and serum level of PB.

Ameliorative effect of dietary probucol on polychlorinated biphenyls-induced hypercholesterolemia and lipid peroxidation in the rat.

The hypocholesterolemic and antioxidant activities of probucol (PB) were examined in rats which were fed for 10 days with diets supplemented with or without 0.02% polychlorinated biphenyls (PCB). Dietary intake of PCB caused multiple effects on lipid metabolism, such as increased levels of both serum and HDL cholesterol, and increased TBA-reactive substances (TBARS), in hepatic subcellular fractions. PB 1% in comparison with either control or alpha-tocopherol (Toc) 0.1%, reduced the serum cholesterol levels in normolipemic as well as in PCB-induced hypercholesterolemic rats. In addition, this drug ameliorated the elevated TBARS induced by PCB in the hepatic subcellular fractions, although less antioxidant activity was noted in rats fed PB than in those fed Toc. The microsomes isolated from various groups were incubated for 2h at 37 degrees C in the presence or the absence of ferrous iron in vitro; microsomes from the PB-fed rats were as much resistant against lipid peroxidation in ferrous-free incubation medium as were those from Toc-fed rats, while in the presence of ferrous iron there was a higher antioxidant effect on lipid peroxidation in the latter fraction than in the former. HPLC analyses showed that less PB than Toc was incorporated into the hepatic subcellular fractions, suggesting that the concentration of antioxidants in hepatic subcellular fractions determine the extent of lipid peroxidation in situ. These results suggest that the administration of PB is an effective approach for the treatment of both hypercholesterolemia and elevated lipid peroxidation while Toc ameliorates only an elevated lipid peroxidation.

A case of Isaacs syndrome with high CSF protein and a large cisterna magna.

An 11-year-old boy exhibited continuous muscle fiber activity. He had suffered from stiffness of his hands, difficulty in relaxing his hands after gripping, and making skilled movements with his fingers. His clinical symptoms improved after treatment with carbamazepine. Electromyography (EMG) showed that he had continuous electrical discharges both at rest and during sleep. These discharges completely disappeared after the peripheral nerve was blocked with Lidocaine. An evoked electromyogram showed suppression of abnormal discharges after the F response. These electrophysiological findings indicated that the disorder originated in the spinal anterior horn cells. CT scanning showed a large cisterna magna in the posterior cranial fossa. Protein in the cerebrospinal fluid was elevated.

[Acute cerebellar ataxia and facial palsy after DPT immunization].

Since the initial report of Beyers & Moll (1948), numerous cases of seizures and encephalopathy after pertussis immunization or DPT immunization have been reported. However, acute cerebellar ataxia and/or facial palsy after DPT immunization is unusual, although there have been several reports from Japan. We report a 1-year-11-month-old girl with acute cerebellar ataxia and facial palsy after DPT immunization. On admission, she was alert. She was active and had a 6-day history of an ataxic gait and asymmetric facial movement which had begun 5 hours after DPT immunization. Neurological examination revealed an ataxic gait, horizontal nystagmus and right facial palsy. A CT scan showed low density on the right side of the pons with marked contrast enhancement. A MRI scan indicated the involvement of not only the right side of the pons, but also of the bilateral cerebellar peduncles. The child did well subsequently and was neurologically normal 20 days after the initial symptoms. To our knowledge, the present case is probably the first reported one of acute cerebellar ataxia after DPT immunization with CT and/or MRI correlation.

High prevalence of infantile autism in Kurume City, Japan.

We surveyed the prevalence of infantile autism in Kurume City. Children born between 1971 and 1979 ranged in age from 4 to 12 years at the time of the survey. We found 51 cases of infantile autism. In each year group, we determined the ratio of the number of cases of infantile autism to the total number of children. The prevalence ranged from 10.4/10,000 to 17.5/10,000, with a mean prevalence of 15.5/10,000. The sex distribution was 42 males (prevalence of 24.8/10,000) and nine females (prevalence of 5.7/10,000). The results showed a significant sex difference, with a male:female ratio of 4:1 (p less than 0.0005). High prevalence rates of infantile autism in Kurume City were confirmed.

Fragile X syndrome in Japanese patients with infantile autism.

Forty-seven patients (39 boys and 8 girls) with infantile autism whose clinical symptoms had matched the diagnostic criteria of DSM III were studied cytogenetically for the occurrence of fragile X [fra(X)] syndrome. The existence of fra(X) chromosome in these patients was screened first by culturing peripheral blood lymphocytes in a medium in which folic acid was absent; the fra(X) chromosome then was confirmed by reculturing in another medium to which 5-fluoro-2'-deoxyuridine was added for the last 24 hours of culture. Fra(X) chromosome was found in 2 of 39 male patients, but in none of the female patients; the 2 patients are siblings. Thus, fra(X) syndrome occurs in 2.6% (1/38) in this study population of male autistic children. The frequencies of fra(X) expression in the older brother with mild mental retardation, in the more severely retarded younger brother, and in their mother were 3-5%, 17-20%, and 9-3%, respectively. Of the two methods used in the present study, the method employing 5-fluoro-2'-deoxyuridine tended to be more sensitive to fra(X) chromosome detection, especially for a suspected carrier.

The prevalence of mental retardation (MR) in Kurume City.

For the purpose of early detection and intervention in mentally handicapped children, the mental retardation (MR) Monitoring Research Committee in Japan initiated a survey in 1981 to determine the prevalence rate and etiology of MR children who had been born between 1969 and 1974, that's those currently from 7 to 12 years of age, living in Kurume City. We found 154 MR children, and that the prevalence ratios in each year group ranged from 5.7/1,000 to 8.1/1,000. The mean prevalence of MR out of all children in each age year group was 7.1/1,000. Of the 154 MR children, 109 were males (9.8/1,000) and 45 females (4.3/1,000), giving a male excess ratio of 2.4:1. The sex difference was significant (p less than 0.005).

A pilot study on the anticonvulsive effects of a thyrotropin-releasing hormone analog in intractable epilepsy.

In 1981, Inanaga et al reported on the efficacy of DN-1417 (a TRH analog) in the treatment of degenerative myoclonus epilepsy and other forms of intractable epilepsy. Following this report, we studied the efficacy and safety of DN-1417 treatment in 10 intractable epileptic children ranging in age from 6 months to 11 years (mean 4 years), including 7 with Lennox syndrome (LS), 2 with West syndrome (WS) and 1 with degenerative myoclonus epilepsy (DME). The daily dose was from 0.02 to 0.05 mg/kg, initially, and then was increased to 0.05 mg/kg. Complete control of seizures was achieved in 2 patients with LS, a 50% or greater decrease in seizure frequency was observed in one patient each with LS and DME and a less than 50% decrease in 1 with LS and 2 with WS. There was no change in 2 LS cases, and 1 LS case became worse. Activation of psychic activities, such as psychomotor activity, facial expression and motivation, was also noted in 7 of the 10 patients. Furthermore, improvement of motor function was noted in 5 patients. Electroencephalographic abnormalities improved in 2 completely seizure free patients with LS in which EEG ameliorated along with clinical seizure control.