Stenting in obstruction of superior vena cava; clinical experience with the self-expanding endovascular prosthesis

From August 1997 to December 2002, 14 consecutive patients with superior vena cava syndrome with the self-expanding endovascular prosthesis. Diagnoses were adenocarcinoma in 6, small cell carcinoma in 4, squamous cell carcinoma in 1, metastatic lung cancer in 2, and invasive thymoma in 1. Atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) were measured on their admission and perioperative period. Expecting only 1 patient complete symptomatically relieved within 3 days of stent implantation. Superior vena cava pressure or radial pressure of the stent was sufficient to relieve obstruction. Preoperative ANP level were normal, BNP level were increased. Postoperatively both ANP level and BNP level were slightly increased under intravenous dopamine hydrochloride. Implantation of the self-expanding stent endovascular prosthesis for superior vena cava syndrome provides rapid symptomatic relief and improves the patient's quality of life.     

A case of well controlled renal small cell carcinoma with metastasis treated by intensive therapy

A 18-year-old male visited hospital with macroscopic hematuria. Computed tomography showed slightly enhanced left renal tumor that was uncharacteristic for clear cell carcinoma, and radical nephrectomy was performed. Operative specimen revealed primary small cell carcinoma of the kidney. 18 months after operation, bone metastases were diagnosed. Chemotherapy including cisplatinum, etoposide and bleomycin and external irradiation was performed. This intensive therapy was effective for metastatic lesion. He is still alive for 55 months after diagnosis, and is the best controlled case around the world. To our knowledge, this is the 14th case as primary renal small cell carcinoma in the world literature.     

D-lactic acidosis in short-bowel syndrome managed with antibiotics and probiotics

D-lactic acidosis sometimes occurs in malabsorbed patients with short-bowel syndrome and is characterized by recurrent episodes of encephalopathy and metabolic acidosis. The characteristic neurologic abnormalities and the presence of metabolic acidosis raises a diagnostic suspicion, and the diagnosis is made when the serum level of D-lactic acid is greater than 3 mmol/L. Standard treatment consists of restricting oral carbohydrates or fasting, correction of metabolic acidosis, and a long-term suppression of pathogenic floras with antibiotics. The authors present a case of D-lactic acidosis in a 22-year-old patient with short-bowel syndrome, to whom intestinal bacterial agents (probiotics) were given in addition to oral kanamycin. Recolonization of the intestine with nonpathogenic floras should be a long-term treatment for D-lactic acidosis.     

Bone marrow chimerism and tolerance induced by single-dose cyclophosphamide

BACKGROUND: Establishment of hematopoietic chimerism is the most stable strategy for donor-specific tolerance. Safer pretreatment regimens are needed for clinical application. We evaluated the efficacy of a simple protocol using cyclophosphamide (CYP) on induction of chimerism and organ transplant tolerance across major histocompatibility complex (MHC) barriers in the rat. MATERIALS AND METHODS: Bone marrow cells from BN (RT1(n)) donors were infused to LEW (RT1(l)) recipients on day 0 after a single injection of CYP at various doses on day -1. Donor-derived hematopoietic chimerism was evaluated by flowcytometry. The recipients received BN or third party (BUF) heart allografts on day 100. RESULTS: While pretreatment with 200 mg/kg of CYP induced high levels of hematopoietic chimerism, six of eight recipients died of severe graft-versus-host-disease (GVHD). CYP at dose of 150 mg/kg induced 36.5 +/- 24.1% of donor-derived chimerism on day 10, and sustained macrochimerism was seen until day 100 without GVHD. Pretreatment with 100 mg/kg of CYP resulted in only transient chimerism (4.8 +/- 5.2%) which disappeared by day 20. In the recipients with 50 mg/kg of CYP, donor bone marrow cells were rapidly rejected and no chimerism was observed. The recipients with 150 mg/kg of CYP accepted BN heart allografts (>100 days x 5), while rejecting BUF allografts by day 12 (n = 4). BN heart allografts were rejected in the recipients with 100 (MST: 57 days, n = 5) and 50 mg/kg (MST: 7 days, n = 5) of CYP. CONCLUSIONS: A single dose of CYP can induce hematopoietic chimerism across MHC-barriers. The dose of 150 mg/kg seems to be optimal to induce organ transplant tolerance without developing GVHD.     

Human parathyroid hormone (1-34) increases mass and structure of the cortical shell,with resultant increase in lumbar bone strength,in ovariectomized rats

Estrogen deficiency causes reduction of bone mass and abnormal bone microarchitecture, consequently reducing bone strength. Human parathyroid hormone (hPTH) (1-34) increases bone mass and strength. To clarify the factors that determine the recovery of bone strength in the lumbar vertebrae of ovariectomized rats by intermittent hPTH administration, we analyzed the relationship between skeletal measurements and bone strength. Human PTH (1-34) administration resulted in recovery of cortical bone mineral content (BMC) and cortical bone area to sham the levels, but in resulted in a less pronounced recovery of trabecular BMC and no increase in the total cross-sectional area of the vertebral body. Of the three-dimensional (3D) trabecular bone parameters, hPTH (1-34) increased trabecular thickness (Tb.Th). The cortical shell area of L4, determined by histomorphometry, was also increased. In hPTH-treated rats, the only determinant of the compressive load of L5 was the cortical shell BMC, in the early recovery period (days 42–84). Our data suggest that increased cortical bone mass contributes more than trabecular bone mass and structure to the recovery of bone strength in response to hPTH therapy in the rat lumbar vertebral body after ovariectomy.     

The W258X mutation in SLC22A12 is the predominant cause of Japanese renal hypouricemia

Recently, a urate transporter, hURAT1 (human uric acid transporter 1) encoded by SLC22A12, was isolated from the human kidney. hURAT1 is presumed to play the central role in reabsorption of urate from glomerular filtrate. In the present study, we analyzed SLC22A12 in seven unrelated Japanese patients with renal hypouricemia whose serum level of urate was less than 1.0 mg/dl, and their family members. We performed direct DNA sequencing of the exon and exon-intron boundaries of SLC22A12 using genomic DNA. Six of the seven patients (86%) possess mutations in SLC22A12. In five patients, a homozygous G to A transition at nucleotide 774 within exon 4 of SLC22A12, which forms a stop codon (TGA) at codon 258 (TGG), was identified (W258X). In one patient, the C to T transition within exon 3, which changes threonine at codon 217 to methionine (T217 M), and the W258X mutation were found (compound heterozygote). Thus, among 12 mutational alleles in six patients, 11 were the W258X mutation (92%). Family members with the heterozygous W258X mutation (carriers) show relatively low levels of serum urate. The present study demonstrates that homozygous W258X mutation is the predominant genetic cause of idiopathic renal hypouricemia in Japanese patients.     

Proximal-type epithelioid sarcoma in a 36-year-old man: closer immunoelectron-microscopic resemblance of the tumor cells to epithelial cells than to mesenchymal cells

Proximal-type epithelioid sarcoma (PES) is a rare neoplasm. We report a case of PES that arose in the perineal subcutis of a 36-year-old Japanese man who died within 4 months of the first clinical sign, probably due to massive pulmonary metastases. In the present study, we analyzed the tumor obtained at surgery, immunohistochemically, immunoelectron-microscopically and genetically. Although the tumor cells in the patient expressed both cytokeratin and vimentin immunohistochemically, they showed epithelial characteristics immunoelectron-microscopically because they had tonofilaments constructed of cytokeratin, not vimentin. In addition, the cytokeratins expressed on the tumor were glandular-type keratins. These findings indicate that PES may be a form of carcinoma in soft tissue. To ascertain the possible origin of the tumor, we compared the tumor immunohistochemically with fetal tissues. Although notochord and fetal peritoneal mesothelium were similar to the tumor antigenically, we could not confirm the specific origin of the tumor. Furthermore, the p53-WAF1 pathway did not contribute to tumorigenesis in the patient because the tumor had no mutation in exons 5-8 of the p53 gene and was immunohistochemically positive for WAF1.     

A 3-year follow-up study of anti-Legionella antibodies in users of Japanese 24-hour hot water baths

Although it has been found that legionellae can exist in a 24-h hot water bath (24HHWB), which has been used recently in Japan, whether longer use of the 24HHWB causes legionellosis is unclear. The present longitudinal study was conducted in 2000 to investigate the 3-yr change in antibody titers in association with the continuous use, non-use, or canceling the use of the 24HHWB, and possible factors relating to the antibody changes. Ninety-two subjects (85 males and 7 females), who had had their anti-Legionella pneumophila (Lp) serum antibody titers measured in our initial study in 1997 and consented to blood sampling 3 yr later, were selected as subjects. There were no clinical cases who had experienced Legionnaires' disease or Pontiac fever during the 3 yr. The continuous users showed no significant changes in antibody titers within 3 yr, whereas the continuous non-users had a significant increase in antibody titers against the Lp serogroup (SG) 5 and 6. Eleven ex-users of the 24HHWB showed a significant decrease in antibody titers against Lp SG 6. The changes in the 24HHWB use, job sector, stress coping strategies, and alcohol-drinking habit were associated with the changes in antibody titers against Lp SG 1, 5 or 6. The anti-Lp antibodies were considered to be IgM dominant. In conclusion, this study indicates that 24HHWB use by healthy subjects does not tend to result in a higher onset risk of legionellosis, even if it is continuously used for 3 yr, although 24HHWB use is likely to induce production of antibodies against legionellae.