Mizoribine in steroid-dependent nephrotic syndrome of childhood

We evaluated a 1-year course of a newly developed immunosuppressant, mizoribine (at a dosage of 3 mg/kg body weight per day), in nine children with steroid-dependent nephrotic syndrome. Steroid treatment could be discontinued in two patients and the maintenance dosage of steroid could be reduced to less than half of that given before mizoribine therapy in a third. There were no beneficial effects in the remaining six patients. No adverse effects of mizoribine were observed during the course of therapy. Received September 20, 1996; received in revised form and accepted April 24, 1997     

Treatment of established osteoporosis with 1α (OH) vitamin D3 and low dose intermittent elcatonin (eel calcitonin derivative)

In addition to estrogen widely used all over the world for the prevention of postmenopausal osteoporosis, calcitonin and vitamin D derivatives are commonly employed to treat established osteoporosis at higher age in Japan. In order to critically assess the usefulness of vitamin D derivatives and calcitonin alone or in combination on the advancement of vertebral deformity at higher age, 32 osteoporotic patients with vertebral deformity with the mean age of 79 were randomly divided into 4 groups with indistinguishable age and severity of the vertebral deformity. Group 1 served as the control without specific medications for osteoporosis. Group 2 was treated with 10 units elcatonin (eel calcitonin derivative) injected intramuscularly twice a week. Group 3 was given 0.75 to 1.5μg/day 1α (OH) vitamin D₃ orally. Group 4 was given a combination of treatments used in Groups 2 and 3. In the lateral X-ray film of the spine taken prior to the test and every 6 months thereafter, the shape of the vertebral body T₈ through L₄ was monitored by measuring the anterior, central and posterior heights. Decrease of the vertebral height ratio; anterior or middle height/posterior or adjacent intact posterior height, by more than 20% of the original value or from above to below 0.80 both appeared to be inhibited during administration of 1α (OH) vitamin D₃. Such effect seems to be augmented by simultaneous administration of elcatonin. Actual decrease of vertebral height ratio values and the per cent fall from the original value significantly less in Groups 3 and 4 than in Group 1. Development of vertebral deformity assessed by the changes of the vertebral height thus appears to decrease during treatment with 1α (OH) vitamin D₃ especially together with calcitonin in established osteoporosis.     

Current role of liver transplantation for the treatment of urea cycle disorders: a review of the worldwide English literature and 13 cases at Kyoto University.

To address the current role of liver transplantation (LT) for urea cycle disorders (UCDs), we reviewed the worldwide English literature on the outcomes of LT for UCD as well as 13 of our own cases of living donor liver transplantation (LDLT) for UCD. The total number of cases was 51, including our 13 cases. The overall cumulative patient survival rate is presumed to be more than 90% at 5 years. Most of the surviving patients under consideration are currently doing well with satisfactory quality of life. One advantage of LDLT over deceased donor liver transplantation (DDLT) is the opportunity to schedule surgery, which beneficially affects neurological consequences. Auxiliary partial orthotopic liver transplantation (APOLT) is no longer considered significant for the establishment of gene therapies or hepatocyte transplantation but plays a significant role in improving living liver donor safety; this is achieved by reducing the extent of the hepatectomy, which avoids right liver donation. Employing heterozygous carriers of the UCDs as donors in LDLT was generally acceptable. However, male hemizygotes with ornithine transcarbamylase deficiency (OTCD) must be excluded from donor candidacy because of the potential risk of sudden-onset fatal hyperammonemia. Given this possibility as well as the necessity of identifying heterozygotes for other disorders, enzymatic and/or genetic assays of the liver tissues in cases of UCDs are essential to elucidate the impact of using heterozygous carrier donors on the risk or safety of LDLT donor-recipient pairs. In conclusion, LT should be considered to be the definitive treatment for UCDs at this stage, although some issues remain unresolved.     

Response of postapoplectic hemichorea/ballism to GPi pallidotomy: progressive improvement resulting in complete relief.

We report on a 66-year-old woman in whom GPi pallidotomy produced progressive and eventually complete relief of hemichorea/ballism (HCB) after a subthalamic hemorrhage. Although choreoballistic movements were unchanged during and immediately after the surgery, the symptoms were gradually improved and consequently abolished by 5 days postoperatively. HCB has never recurred up to the present (9 months follow-up period). This note is the first report describing detailed postsurgical process in HCB relief after pallidotomy.     

Open heart surgery in a paraplegic patient.

We operated on a patient who had been paraplegic since sustaining a spinal cord injury 11 years ago. We made a reversed L-shaped sternum incision and cannulated all tubes for the cardiopulmonary bypass through a wound window. This provided an excellent surgical view without restricting the patient's upper limbs (needed for wheel chair operation), and recovery was good. Just after surgery, however, it was difficult to control blood pressure and the loss of serum albumin. We believe this is the first report of open-heart surgery undertaken in a paraplegic patient and that the reversed L-shaped incision and careful monitoring of hemodynamics each played an important role in the successful outcome. We hope that this report will help in the treatment of other paraplegic patients who need open-heart surgery.     

Histochemical study of mitochondrial enzymes in cerebellar cortex of macular mutant mouse,a model of Menkes kinky hair disease

The cerebellar Purkinje cells in the hemizygote of the macular mutant mouse contain numerous abnormal mitochondria which show a marked decrease in cytochrome c oxidase activity. Using histochemical methods we studied the activity of other mitochondrial enzymes, such as NADH diaphorase and succinic dehydrogenase, in the cerebellar cortex of this mutant mouse. Such activities were markedly increased in the Purkinje cells, especially in the soma and stem dendrite, from 10 days after birth in the hemizygote as compared with findings in normal littermates. These results were considered to be due to an increased number of abnormal mitochondria.Supported by the Research Grants nos. 2A-5-03 and 3B-1-04 for Nervous and Mental Disorders from the Ministry of Health and Welfare, and by Grant no. 04670492 of the Ministry of Education of Japanese Government     

Assessment of skull base involvement of nasopharyngeal carcinoma by bone SPECT using three detectors system]

SPECT of the skull was performed on twelve patients with histologically proven nasopharyngeal carcinoma using newly developed three detectors SPECT system (Toshiba GCA 9300A) mounted with fan-beam collimators, for the purpose of early detection of skull base involvement of the disease. This SPECT system has extremely improved resolution with FWHM of 8 mm in the center, and it provides clear tomographic images of the skull which has anatomically complex structure. SPECT image was taken 3 hours after injection of 740 MBq (20 mCi) of 99mTc-MDP following whole body skeletal survey. In twelve patients with confirmed nasopharyngeal carcinoma, 8 patients showed positive findings on SPECT. Three of SPECT positive patients also showed destructive findings on CT. Other five positive patients did not show destructive findings on CT at their initial examinations, but in three of them CT findings turned to positive later. SPECT was superior to CT in early detection of skull base involvement. We believe that SPECT of the skull is a diagnostic tool for early detection of skull base involvement of nasopharyngeal carcinoma.     

Biotype traits and antibiotic susceptibility of Vibrio cholerae serogroup O1 before, during and after the emergence of the O139 serogroup.

Sixty-nine strains of Vibrio cholerae O1 isolated at different times were analysed to investigate if there were any differences among the O1 strains isolated before, during and after the advent of the O139 serogroup. Of the 69 O1 strains examined, 68 belonged to the Ogawa serotype while one belonged to the Inaba serotype. With the exception of one strain all other strains of V. cholerae O1 belonged to the eltor biotype. A single O1 strain isolated before the emergence of the O139 serogroup could not be classified as either eltor or classical biotype because it was resistant to both classical and eltor specific bacteriophages. Marked variations in the susceptibility to antibiotics of V. cholerae O1 isolated during the different periods were observed. In addition, strains of V. cholerae isolated after the epidemic of serogroup O139 in Calcutta showed an expanding R-type with resistance to a variety of drugs as compared to the O1 strains isolated before the advent of the O139 serogroup. From this study, it is clear that there is a substantial mobility in genetic elements of V. cholerae O1 which necessitates a continuous monitoring to keep abreast of the changing traits of the etiologic agent of cholera.