CONGENITAL NEPHROTIC SYNDROME (FINNISH TYPE)

The case was a one year and four months old boy, who had been diagnosed as congenital nephrotic syndrome on the 4th day of life. He died of septicemia from peritonitis. Large pale kidneys, dilated trunk of pulmonary artery, and thickened left atrial endocardium were observed at autopsy. Renal histology seemed to be compatible with congenital nephrotic syndrome of the Finnish type. Viral antigens of herpes simplex type 1 and varicella-zoster in paraffin-embedded sections were proven to be negative. Besides renal change, increased number of pancreatic islets without hypertrophy was noticed. ACTA PATHOL. JPN. 35: 517–525, 1985.     

Multiple basilar trunk aneurysms and bilateral internal carotid artery hypoplasia

We describe a case of a 32-year-old woman with bilateral hypoplastic internal carotid arteries who presented with subarachnoid haemorrhage and was found to have two basilar trunk aneurysms. Carotid angiography showed that both internal carotid arteries were narrowed at their origin and terminated at the cervical segment. Vertebral angiography demonstrated a dilated vertebrobasilar system which supplied sufficient blood flow for both cerebral hemispheres through the posterior communicating arteries. The patient was successfully treated by multiple operations.     

Effects of opening peritumoral syrinxes on motor evoked potentials during resection of an intramedullary spinal cord tumor--case report

A 29-year-old woman presented with mild tetraparesis caused by an intramedullary cervical cord tumor extending from the C-5 to C-6 levels associated with rostral and caudal syrinxes extending to the C-1 and C-7 levels. Transcranial motor evoked potential (MEP) monitoring was carried out during tumor resection using high-frequency repetitive electrical stimulation. When the rostral syrinx was opened, the MEP amplitude in the right upper limb suddenly decreased by about 50%. When the caudal syrinx was opened, the amplitude immediately recovered to the baseline. During dissection of the attachment of the tumor, the amplitude decreased again by 70%, and did not recover. The tumor was totally removed. The histological diagnosis was ependymoma. Postoperatively, motor weakness in the right arm deteriorated but gradually recovered. The initial loss of amplitude on opening of the rostral syrinx may have resulted from distortion of the intramedullary pressure that compromised function of the motor tract nearby. Opening of the caudal syrinx normalized the intramedullary pressure and allowed the MEP to recover. Intraoperative monitoring of MEPs is valuable in minimizing injury to corticospinal pathways, but care must be taken in evaluating changes in MEP. Intramedullary spinal tumors with associated syrinxes are not rare, so surgeons should realize that opening of a syrinx can lead to MEP changes.     

Dominant negative protein kinase Cbeta improves 1alpha, 25-dihydroxy vitamin D3-induced insulin resistance

1alpha,25-Dihydroxy vitamin D3 (1,25D3) activates conventional PKC and may subsequently lead to insulin resistance. Previous studies from our laboratory have shown that pretreatment with 10 nM-10 microM 1,25D3 dose-responsively suppressed insulin-induced glucose. To assess PKC(beta)-mediated inhibition of insulin-induced glucose uptake in rat adipocytes, we preincubated with Go6976 and LY379196, conventional PKC inhibitors, and found they abolished the 1,25D3-mediated inhibitory effect on insulin-induced 2-deoxyglucose (DOG) uptake. Moreover, the inhibitory effect of 1,25D3 on insulin-induced DOG uptake was abrogated in adipocytes overexpressed with dominant negative PKC(beta), but not in those overexpressed with wild type PKC(beta). These results suggest that 1,25D3 reduces insulin-induced glucose uptake via activation of PKC(beta) in rat adipocytes.     

Familial dysalbuminemic hypertriiodothyroninemia in a Japanese kindred

A 56-year-old Japanese housewife had been diagnosed as having Graves' disease and was treated with methimazole. When she was referred to our hospital, the serum T3 level was high irrespective of high TSH level. High serum T3 levels were also observed in two out of her three sisters. Electrophoresis revealed that binding of 125I-T3 to serum albumin was markedly increased whereas the binding of 125I-T4 to serum albumin was slightly increased in the three sisters whose serum T3 levels were high. These data indicate that the presence of an albumin variant is the cause of hypertriiodothyroninemia in this family.     

Expression profiles of the duplicated matrix metalloproteinase-9 genes suggest their different roles in apoptosis of larval intestinal epithelial cells during Xenopus laevis metamorphosis.

Matrix metalloproteinases (MMPs) play a pivotal role in development and/or pathogenesis through degrading extracellular matrix (ECM) components. We have previously shown that Xenopus MMP-9 gene is duplicated. To assess possible roles of MMP-9 and MMP-9TH in X. laevis intestinal remodeling, we here analyzed their expression profiles by in situ hybridization and show that their expression is transiently up-regulated during thyroid hormone-dependent metamorphosis. Of interest, MMP-9TH mRNA is strictly localized in the connective tissue and most highly expressed just beneath the larval epithelium that begins to undergo apoptosis. On the other hand, cells expressing MMP-9 mRNA become first detectable in the connective tissue and then, after the start of epithelial apoptosis, also in the larval epithelium. These results strongly suggest that MMP-9TH is responsible in the larval epithelial apoptosis through degrading ECM components in the basal lamina, whereas MMP-9 is involved in the removal of dying epithelial cells during amphibian intestinal remodeling.     

Associations between physical strength, cerebral function and mental health in independent-living elderly Japanese women

Objectives  To evaluate the physical strength, cerebral function, and mental health conditions in elderly persons, and to examine the correlation between these functions. Methods  The subjects were 151 independent-living elderly Japanese women, mean age 70 +/-5 years (+/-SD; range, 60 to 80 years), with normal abilities in daily life. The health check-up was conducted from April to May 1997. Physical strength was estimated by measuring seven activities. Cerebral functions were assessed by six sub-tests of the General Aptitude Test Battery (GATB). Their mental health status was measured by four sub-scales of the General Health Questionnaire (GHQ)-28. Results  The physical strenght and almost all of the cerebral functions decreased with age. Social dysfunction and severe depression on the GHQ sub-scales also worsened with age. Physical strength was strongly correlated with cerebral functions after adjusting for the confounding effect of aging. There were also interrelations between physical strength and mental health. Conclusions  This study provided important information on the correlation between physical and mental status in elderly women. Future longitudinal studies with the intervention of physical training are required to determine whether a causal relationship exists between these factors.     

Effect of distributing an evidence-based guideline for prevention of osteoporosis on health education programs in municipal health centers: a randomized controlled trial

Background

Current health education programs for osteoporosis prevention are not strictly evidence-based. We assessed whether distribution of an evidence-based guideline improved such programs at municipal health centers.

Methods

This randomized controlled trial evaluated 100 municipal health centers throughout Japan that were randomly selected from those that planned to revise osteoporosis prevention programs. The implementation status of educational items recommended by the guideline was assessed before and after the intervention by evaluators blinded to the allocation. After the pre-intervention assessment, centers were randomly allocated in a 1:1 ratio to intervention and control groups by a minimization method defining region and city/town as stratification factors. Centers in the intervention group were given copies of the guideline; centers in the control group were instructed to use any information except the guideline. Analyses were performed on an intention-to-treat basis.

Results

The guideline was used by 50% of the intervention group. Before the intervention, there was no significant difference in the evidence-based status of health education between the groups. The post-intervention assessment showed that the implementation rates of health education on dietary calcium intake for postmenopausal women and exercise for elderly persons were higher in the intervention group. Specific advice on intakes of calcium and vitamin D and exercise became more evidence-based in the intervention group.

Conclusions

The findings suggest that the guideline helped healthcare professionals to improve health education programs by making them more evidence-based. However, the improvements seemed to be limited to items that the professionals felt prepared to improve.Key words: evidence-based guideline, fracture, health education, osteoporosis, randomized controlled trial     

Quantitative analysis by digital computer of 99mTc-N-pyridoxyl-5-methyltryptophan (99mTc-PMT) hepatogram in diffuse parenchymal liver diseases

99mTc-N-pyridoxyl-5-methyltryptophan (99mTc-PMT) hepatograms were analyzed to provide information about the liver and bile duct. Calculations were based on a four compartmental model and included corrections for blood, tissue, hepatic parenchymal and bile backgrounds. The time activity curves for 99mTc-PMT in the cardiac region were described as the sum of two exponential functions, while curves for the hepatic regions were described as the sum of three exponential components. The measured hepatograms were compared with simulations and good agreement between the two curves showed that the compartmental model adequately described the blood and bile activities in vivo. Hepatic excretion rate constants were 0.179 +/- 0.026 in three normal subjects, 0.102 +/- 0.012 in four patients with chronic hepatitis and 0.082 +/- 0.034 in six patients with liver cirrhosis. In the case of diffuse parenchymal liver disease, there were lower rate constants for the excretion from the liver to the bile ducts than in normals, and the relative distribution volumes were also larger than normal. Prior to the development of this compartmental model, no useful kinetic model had been found which could satisfactorily explain the time activity curves. Experience in human studies proves this method to be accurate in determining the rate constants for the hepatobiliary transport of 99mTc-PMT.