Tonsillectomy and steroid pulse therapy for recurrent IgA nephropathy in renal allograft.

We experienced two cases of steroid pulse therapy combined with tonsillectomy for recurrent IgA nephropathy (IgAN) in a renal allograft. We defined recurrent IgAN in renal allograft as IgA deposits in glomeruli with persistent proteinuria (> 0.5 g/ day) and microscopic hematuria in renal transplant recipients with biopsy-proven IgAN of their native kidneys. We performed steroid pulse therapy following tonsillectomy as therapeutic protocol for recurrent IgAN. The first patient was diagnosed with recurrent IgAN by allograft biopsy 3 years after renal transplantation, and a second patient was diagnosed after one year. The former patient's proteinuria disappeared 4 months after treatment and the latter patient's proteinuria disappeared after one month. Tonsillectomy combined with steroid pulse therapy can induce clinical remission in patients with recurrent IgAN after renal transplantation.

[Rupture of intrascrotal epidermoid cyst complicated by bacterial infection: a case report].

A 63-year-old male visited our hospital with the chief complaint of right scrotal pain. The right scrotum was swollen to the size of a small egg, and its skin was reddish. The mass was palpable independent of the right testis and epididymis. We diagnosed an intrascrotal abscess. The pus spontaneously issued from the scrotal mass. Sequentially, the abscess was extracted under spinal anesthesia. Membrane-like tissue assumed as the abscess wall was removed. Histologically, the abscess wall was composed of epidermal structure with epidermal keratinization, and horny material was found inside the wall. In the scrotal epidermis overlying the abscess, infiltration of neutrophils, lymphocytes, and multinucleated giant cells were observed. Anaerobic bacteria were detected in the pus of the abscess. Consequently, we diagnosed this case as rupture of an intrascrotal epidermoid cyst complicated by bacterial infection.

Mechanisms of the antimetastatic effect in the liver and of the hepatocyte injury induced by alpha-galactosylceramide in mice.

The role of mouse liver NK1.1 Ag(+) T (NKT) cells in the antitumor effect of alpha-galactosylceramide (alpha-GalCer) has been unclear. We now show that, whereas alpha-GalCer increased the serum IFN-gamma concentration and alanine aminotransferase activity in NK cell-depleted C57BL/6 (B6) mice and B6-beige/beige mice similarly to its effects in control B6 mice, its enhancement of the antitumor cytotoxicity of liver mononuclear cells (MNCs) was abrogated. Depletion of both NK and NKT cells in B6 mice reduced all these effects of alpha-GALCER: Injection of Abs to IFN-gamma also inhibited the alpha-GalCer-induced increase in antitumor cytotoxicity of MNCS: alpha-GalCer induced the expression of Fas ligand on NKT cells in the liver of B6 mice. Whereas alpha-GalCer did not increase serum alanine aminotransferase activity in B6-lpr/lpr mice and B6-gld/gld mice, it increased the antitumor cytotoxicity of liver MNCS: The alpha-GalCer-induced increase in survival rate apparent in B6 mice injected intrasplenically with B16 tumor cells was abrogated in beige/beige mice, NK cell-depleted B6 mice, and B6 mice treated with Abs to IFN-gamma. Depletion of CD8(+) T cells did not affect the alpha-GalCer-induced antitumor cytotoxicity of liver MNCs but reduced the effect of alpha-GalCer on the survival of B6 mice. Thus, IFN-gamma produced by alpha-GalCer-activated NKT cells increases both the innate antitumor cytotoxicity of NK cells and the adaptive antitumor response of CD8(+) T cells, with consequent inhibition of tumor metastasis to the liver. Moreover, NKT cells mediate alpha-GalCer-induced hepatocyte injury through Fas-Fas ligand signaling.

Step by step filter based program for calculations of highly informative derivative curves

We have developed a microcomputer program, based on a recently described approach called step by step filter (SBSF), for calculation of derivative curves directly from spectra recorded as a function of wavelength. This program avoids the long wavelength attenuation featured at conventional method for derivative curves calculation, and in this extent could be very helpful for daily spectroscopy practice. The features of the SBSF program include: easy treatment of data through a windowed environment, calculating of both conventional and step by step filter derivatives, possibilities for selection of the mathematical conditions for smoothing and differentiation simultaneous plotting of the original curve and its derivative and a mouse pointer. Several examples from different branches of the molecular spectroscopy (absorption UV-VIS, CD and fluorescence) are provided and discussed in the terms of advantages of SBSF.

[A clinical study on active vitamin D pulse therapy in hemodialysis patients with secondary hyperparathyroidism].

The oral active vitamin D pulse therapy was performed for 20 chronic hemodialysis patients with secondary hyperparathyroidism (2 degrees HPT). Before pulse therapy, all patients showed high-turnover bone diseases, elevated serum alkaline phosphatase (Alp) and elevated serum parathyroid hormone (PTH). They had at least one parathyroid gland detected by ultrasonography. In one patient, serum PTH level did not decrease during three months. In five patients, hypercalcemia was observed so that the pulse therapy was with drawn. Another patient was resistant to the second pulse therapy for recurrence of 2 degrees HPT. Total parathyroidectomy and autotransplantation was performed in these seven patients. The size of parathyroid gland in patients undergoing surgical treatment was significantly larger than those estimated by ultrasonography in patients successfully treated with pulse therapy (p < 0.005). In all patients with surgical treatment, the conventional oral active vitamin D therapy was performed with caution to hypercalcemia preoperatively. The preoperative Alp levels decreased compared with those of at the beginning of the pulse therapy. Postoperatively, the total amount of intravenous administered calcium decreased, and serum calcium levels were easily controlled.

Increased activity and intranuclear expression of phospholipase D2 in human renal cancer.

We examined the PLD activities of human renal cancers and found that the PLD2 activity was greatly elevated in almost all cases examined as compared with the adjacent normal region. Western blot analysis showed the increased levels of PLD2 protein, but the PLD1 was not discernible. The oleate-dependent PU) activity was very low but appeared to increase in most cases. Interestingly, the immunohistochemical observations indicated the high expression of PLD2 in the nuclei of clear carcinoma cells. This is the first demonstration which suggests the possible involvement of PLD2 in tumorigenesis of renal cancer.

Loss of tissue immunoreactive androgen receptor in prostate cancer presenting initially as an intracranial tumor.

We report a case of prostate cancer that presented initially as an intracranial tumor. Biopsy specimens of the prostate before endocrine treatment were nearly negative immunohistochemically for prostate-specific antigen and the androgen receptors. All metastases including those in the brain expressed neither androgen receptor nor prostate-specific antigen at the protein and mRNA levels. The tumor, which did not respond to the anti-androgen therapy, had an aggressive course. In this case, the androgen-independent growth and rapid progression might be associated with the initial loss of the antigen and androgen characteristics of the prostate.

A case in the spectrum of the oculo-encephalo-hepato-renal syndrome.

An 18-year-old male is presented with unprecedented central nervous system findings (cerebral dysplasia and sacral meningocele) possibly in the spectrum of the oculo-encephalo-hepato-renal syndrome. He had severe mental retardation, triplegia, epilepsy, retinitis pigmentosa, and chronic renal failure. Magnetic resonance imaging demonstrated cerebral dysplasia (left dominant abnormal gyri, hypoplastic white matter, basal ganglia, and thalamus, and absence of the septum pellucidum) and the hypoplastic cerebellum and brainstem. A sacral meningocele was observed first at 16 years of age. His renal function gradually worsened after 11 years of age. His liver function was normal. The previously reported 72 cases with the oculo-encephalo-hepato-renal syndrome are reviewed.

[Bacillus Calmette-Guerin intravesical instillation treatment for carcinoma in situ of the bladder. Gifu BCG Instillation Therapy Research Group].

We performed a retrospective long-term study to evaluate the efficacy of intravesical instillation of Tokyo 172 strain Bacillus Calmette-Guerin (BCG) on carcinoma in situ (CIS) of the bladder. Between 1989 and 1998, 42 patients with CIS of bladder underwent intravesical instillation of BCG. In the follow-up period from 6 to 116 months (mean: 37.3 months), the efficacy rate of intravesical BCG instillation for CIS of the bladder was 81.0%. Two patients died from the bladder cancer. The non-recurrence rate in patients with grade 2 carcinoma (19 cases) was not significantly different from that in those with grade 3 carcinoma (23 cases). However, the recurrence rate in patients with secondary CIS (15 cases) was significantly higher than in those with primary CIS (27 cases). The recurrence of CIS was observed in 7 of 42 cases. In 6 of 7 patients, CIS recurred within one year after treatment. Total cystectomy was performed in 10 of 42 patients, and pathological findings of muscle layer invasion were detected in 8 patients. Although side effects occurred in 33 patients (77.5%), no clinically significant side effects were observed. Our results suggest that intravesical BCG therapy may be useful for the treatment of CIS of the bladder.

[The current status of molecular detection of micrometastatic prostate cancer cells].

We summarized and reviewed published reports, including our studies, on the reverse transcription-polymerase chain reaction (RT-PCR) detection of micrometastatic prostate cancer cells in lymph nodes, bone marrow and peripheral blood. Some published data preliminarily suggest that the RT-PCR assay of micrometastatic prostate cancer cells may allow a more accurate assessment of lymph node and bone metastases of prostate cancer, and offer a presurgical prediction of the pathological stage of clinically localized disease. In addition, the RT-PCR assay may have a unique prognostic value in prostate cancer. However, controversy remains over the clinical significance of the RT-PCR assay. This assay could potentially develop into a diagnostic procedure for the clinical decision making in patients with prostate cancer. To establish the clinical significance of the RT-PCR assay, further optimized and standardized RT-PCR assay studies are needed, investigating large populations and involving long-term follow-up for the determination of any association between the results of the RT-PCR assay and specific clinical outcome.

Synthesis of NBD-alpha-galactosylceramide and its immunologic properties.

[formula: see text] A representative alpha-galactosylceramide (alpha-GalCer), KRN7000, can activate NKT cells through CD1d molecules, which play an essential role in the generation of the strong antitumor activity of KRN7000. Our previous study has demonstrated that alpha-GalCer binds directly to CD1d molecules. However, it is controversial whether CD1d binds alpha-GalCer in endosomal compartments. To address this question, we synthesized NBD-alpha-GalCer, which has strong fluorescent properties. We found that the NBD-alpha-GalCer has immunostimulatory activity that is stronger than that of KRN7000.

Effects on growth of rat offspring born from dams treated subcutaneously with a surfactant, polyoxyethylene(10)nonylphenyl ether(NP-10), during lactational period.

A surfactant NP-10 was administered subcutaneously to Jcl:Wistar female rats at dose levels of 5, 20 and 80 mg/kg/day from date of birth to day 21 after birth of F1 offspring to assess its effects on the growth, behavior and functions of the offspring. For F0 dams, scab formation and loss of hair at the test substance administration site were observed in all treatment groups and induration of the skin at the test substance administration site in the 20 and 80 mg/kg groups in general condition and necropsy findings at the end of the dosing period. In necropsy findings, in addition to these changes, hemorrhage and whitish change of the subcutis at the test substance administration site were seen in all treatment groups, adhesion to the somatic muscles and granulation of the subcutis at the test substance administration site in the 20 and 80 mg/kg groups, and swelling of the spleen and adrenals in the 80 mg/kg group. Reduction or a tendency for reduction in food consumption was also detected from the initial day of dosing (day 0) to day 17 after birth F1 offspring in the 80 mg/kg group. Body weight or the findings on the day after birth and day of weaning failed to reveal any evidence of an effect that could be ascribed to the test substance. In F1 born offspring, a decrease or tendency for decrease in body weight was observed from day 7 after birth in both sexes and for females during the gestation period in the 80 mg/kg group. However, body weight gains based on the weights at 4 weeks after birth or on day 0 of gestation in the 80 mg/kg group failed to reveal any difference from that in the control group. The observation on the day of birth and during the period of lactation, physical development test, reflex test, general condition, open-field test, water-maze test, reproductive ability test, observations at cesarean section, necropsy findings, organ weights, histopathological findings of females or males that did not achieve successful gestation, skeletal examination, and the observations at cesarean section and external examination of F2 fetuses failed to reveal any evidence that could be ascribed to the test substance. These results indicate that NP-10 had no effect on the behavior or functions of the offspring, although it affected the growth of the offspring born, under the conditions of this study. The non-effective dose level is considered to be 20 mg/kg for general toxicity of the dams and for their offspring.

Neuroleptic malignant syndrome and methylphenidate.

A 1-year-old female presented with neuroleptic malignant syndrome probably caused by methylphenidate. She had defects in the supratentorial brain including the basal ganglia and the striatum (multicystic encephalomalacia) due to severe perinatal hypoxic-ischemic encephalopathy, which was considered to be a possible predisposing factor causing neuroleptic malignant syndrome. A dopaminergic blockade mechanism generally is accepted as the pathogenesis of this syndrome. However, methylphenidate is a dopamine agonist via the inhibition of uptake of dopamine, and therefore dopaminergic systems in the brainstem (mainly the midbrain) and the spinal cord were unlikely to participate in the onset of this syndrome. A relative gamma-aminobutyric acid-ergic deficiency might occur because diazepam, a gamma-aminobutyric acid-mimetic agent, was strikingly effective. This is the first reported patient with neuroleptic malignant syndrome probably caused by methylphenidate.

Epidemiology of spina bifida in Tottori Prefecture, Japan, 1976-1995.

The authors studied the epidemiology of spina bifida in Tottori Prefecture, Japan, from 1976 to 1995. Thirty-four patients (16 men and 18 women) were registered in this study. Consanguineous marriages, familial occurrence, and abnormalities in prenatal history were not observed. The incidence rate in the entire prefecture and in the eastern, central, and western regions was 0.234, 0.148, 0.425, and 0.230 per 1,000 live births, respectively. The incidence rate in the central region was greater than that in the eastern region with statistical significance (P < 0.05), but the cause of the cluster is unknown. The incidence rate of 0.234 per 1,000 live births for 20 years is compatible with the previous two studies of 1922-1940 and 1948-1954 in Japan. Such apparently stable trends suggest that environmental factors have affected the Japanese less than genetic factors. Seasonal variations are not demonstrated.

New syndrome with the Sakoda complex, bilateral anophthalmia, and cortical dysgenesis.

An 8-year-old Japanese boy had Sakoda complex (basal encephalomeningocele, agenesis of the corpus callosum, and cleft lip and/or palate) associated with bilateral anophthalmia, dysgenesis of the cerebral cortex, severe mental retardation, and intractable epilepsy as core symptoms and hemiparesis, microcephalus, short stature, and hemivertebra. Tada and Nakamura described the first case of the Sakoda complex associated with bilateral anophthalmia, cortical dysgenesis, neonatal-onset seizures, and severe mental retardation. Fourteen patients with the Sakoda complex with or without ocular dysplasia were reviewed. It is proposed that these cases belong to a clinical entity that is distinguishable from the remaining 12 patients because of bilateral anophthalmia, cortical dysgenesis, and its resulting severe mental retardation and intractable epilepsy. There is a possibility that these two cases are one severe end of certain spectrum disorders in which certain common gene(s) might be implicated.