Analysis of the hemostasis system using biochemical techniques in children with minimal change nephrotic syndrome(MCNS) has previously been restricted to in vitro assays. The recent introduction of measurement of shear stress-induced platelet aggregation(SIPA) using platelet-rich plasma(PRP) has facilitated detailed investigation of the hemostatic system in vivo. In order to further analyze the etiology of the thrombotic tendency exhibited by patients with childhood MCNS, we investigated SIPA at both low shear stress (L-SIPA) and high shear stress (H-SIPA) in 14 children with MCNS using PRP collected weekly after commencing prednisolone therapy. Seven patients were newly diagnosed cases of MCNS (ND) whereas the remainder had suffered a disease relapse (DR) . Prior to prednisolone therapy L-SIPA, which was thought to be affected by fibrinogen (Fbg) levels, was significantly increased in both patient groups compared to normal controls (17.4±4.1 % vs. 3.6±0.7 %, ND vs control, p< 0.01 : 11.7±3 % vs. 2±0.7 %, DR vs control, p<0.01) with values declining at weekly intervals thereafter. Plasma Fbg levels in simultaneously collected samples followed a similar course. In contrast, H-SIPA, which was thought to be affected by von Willebrand factor (VWF), was significantly enhanced in MCNS patients after 1 week of prednisolone therapy compared to the controls (45±5.1 % vs. 26.3±3.5 %, ND vs normal, p<0.05 36.9±3.3 % vs. 25.5±1.6 %, DR vs. normal, p<0.05). Plasma ristocetin cofactor and VWF antigen levels in simultaneously collected samples followed a similar course, whereas thrombin-antithrombin complex (TAT) levels remained constant. These results indicate that SIPA is enhanced in the acute stage of childhood MCNS, especially L-SIPA prior to the initiation of prednisolone therapy and H-SIPA after 1 week of prednisolone therapy, and that these phenomena may be affected by plasma Fbg and VWF levels, respectively. The enhanced SIPA may play an important thrombogenic role in the acute phase of childhood MCNS.

View full abstract

Background : It is well recognized that parenteral iron administration is recommended for hemodialysis (HD) patients treated with rHuEPO. On the other hand, hepatic iron concentration increases in chronic hepatitis C, and iron reduction improves serum transaminase levels in these patients.Methods : We compared the rHuEPO doses with hematological parameters in HCV positive (n=7) and HCV-negative (n=32) HD patients when parenteral low-dose iron was administered for one year (target ferritin level : 200-300ng/ml, target hematocrit level : 30-33 %).Results : None of the parameters was significantly different between the groups at the start of the study. One year later, levels of hematocrit and serum ferritin significantly increased compared with those at the start in each group (HCV-positive group : 28.0±2.7→31.3±1.5 %, p< 0.01, 119.3±171.9→303.3±77.7 ng/ml, p<0.05, respectively, HCV-negative group : 26.8±2.2→30.0±3.5 %, p< 0.01, 69.8±100.5→278.4±66.4 ng/ml, p<0.01, respectively) . Serum transaminase levels were not significantly different between the start and end points in the HCV-positive group, but 2 patients showed an increase in these levels. In the HCV-negative group, the rHuEPO dose at the end point was significantly reduced compared with that at the start (4, 875±2, 089→4, 031±2, 203 IU/W, p<0.05). In the HCV-positive group, however, it was difficult to reduce the rHuEPO dose in order to maintain the target hematocrit level (4, 071±1, 134→3, 857±1, 464IU/W, NS).Conclusion : We suggested that rHuEPO should be used together with parenteral iron administration, even in HCV-positive HD patients, because it is safe at low doses under careful observation.

View full abstract

The pathophysiology of encapsulating peritoneal sclerosis (EPS) that develops after withdrawal from long-standing dependence on CAPD remains unclear. The aim of this study was to clarify the risk factors for EPS as expressed in the peritoneal function. Fourteen patients who had shifted to standard hemodialysis after long-term CAPD (average, 105 months) were studied : 3 developed EPS after PD withdrawal while 11 did not. Analysis of the data obtained from the peritoneal equilibration test (PET) revealed that : (1) the dialysate/plasma creatinine ratio (D/Pcr) was significantly higher in the EPS group than in the non-EPS group during the course of PD as well as after PD withdrawal ; and (2) eight patients, including the 3 with EPS, were classified as being in a high-transport membrane state (HTS) at PD withdrawal. The duration of HTS during PD was longer in those patients with EPS. During the periods after PD withdrawal, none of these EPS patients recovered from HTS, whereas 4 of the 5 non-EPS patients did. These data suggest that long-standing HTS during the course of PD as well as post withdrawal, may be risk factors for EPS development. For this reason, it is indicated that PET has clinical relevance in examining sequential changes in peritoneal function and in detecting those patients at risk of EPS.

View full abstract

Fluvastatin, an HMG-CoA reductase inhibitor, was administered at a dosage of 20 mg/day for 24 weeks to 11 hemodialysis patients with a high plasma total cholesterol (TC) level(≥220 mg/dl) . Serum lipids, apolipoprotein, and malondialdehyde (MDA) levels were measured every 12 weeks. After 24 weeks of fluvastatin administration, the TC level had decreased by 10.5 %(238±15 mg/dl→→203±25mg/dl), the low density lipoprotein cholesterol (LDL-C) level had decreased by 16.2 %(142±32mg/dl→119±26 mg/dl), and the HDL-C level had increased by 23.4 %(47±15mg/dl→58±19 mg/dl). These changes were statistically significant and resulted in a reduction of the atherogenic index (AI : TC-HDL-C/ HDL-C) . The triglyceride (TG) level did not change significantly. The apolipoprotein Al level increased by 9.1 %(121±22 mg/dl→132±20 mg/dl) and the apolipoprotein B level decreased by 20.2 %(114±25 mg/dl→91±20 mg/dl). The MDA level also decreased significantly(1.16±0.92 nmol/ml→0.58±0.38 nmol/ml) . No particular side effects were observed during the 24 weeks of fluvastatin administration. In conclusion, fluvastatin may play an important role in preventing significant oxidative stress and was shown to be safe and effective in reducing the TC, LDL-C, MDA and AT levels in dialysis patients with hypercholesterolemia. The possibility that this improvement in the plasma lipid profile of dialysis patients may decrease atherogenic complications requires further investigation, including long-term clinical observations.

View full abstract

Secondary hyperparathyroidism (II HPT) is a major complication in chronic dialysis patients, and percutaneous ethanol injection therapy (PEIT) has become a useful alternative treatment for II HPT. However, the existence of ectopic parathyroid glands is a major problem when conducting PEIT. Ectopic parathyroid gland accepts 10-35 % of IIHPT, and the missing glands cannot be detected consistently by any imaging techniques, including scintigraphy. Intrathyroid parathyroid gland is as rare as about 1% and recurrence of missing glands after parathyroidectomy (PTx) has been reported in some cases. We report here a 52-year-old female in whom an ectopic parathyroid gland was defected successfully and intact-PTH controlled by tentative PEIT. At the first examination, a left parathyroid adenoma and a right thyroid goiter were pointed out by ultrasonography, CT and scintigraphy. PEIT was applied twice to the left parathyroid adenoma, but intact-PTH was not decreased. Ultrasonography, CT, ²⁰1T1^-99mTc subtraction scintigraphy and fine needle aspiration biopsy (FNAB) were performed again to search for the exsistence of ectopic glands. The results suggested that the right intrathyroid tumor was an ectopic parathyroid gland. Consequently, tentative PEIT was applied to the right intrathyroid tumor, and successful control of intact-PTH and serum Ca was eventually achieved. To our knowledge, this is the first reported case of secondary hyperparathyroidism with an ectopic intrathyroid gland that was successfully controlled by PEIT. In this case, it was suggested that tentative PEIT of intrathyroid tumor was a useful method for detecting an ectopic parathyroid gland.

View full abstract

This report concerns the first case in Japan of interstitial nephritis induced by mesalazine, a new therapeutic agent for inflammatory bowel disease, such as ulcerative colitis. Twenty-two cases have already been reported in other countries. The patient, a 27-year-old woman, was treated with mesalazine for her ulcerative colitis at another hospital. At the beginning of her treatment, her serum creatinine level was within the normal range. After 12 months, this level increased up to 5.7 mg/dl. She was then referred to our hospital for renal investigation and therapy. A renal biopsy revealed that severe tubulo-interstitial nephritis had occurred. Her mesalazine treatment was withdrawn and prednisolone was administered. Her serum creatinine level decreased gradually. However, this level remained at about 2.8 mg/dl and stabilized at that level. She was then discharged from the hospital. Glomeruli appeared to have minor glomerular abnormalities except for one globally sclerosed glomerulus as observed by light microscopy. However, IgM and C3 deposition on glomeruli were also observed. Glomerular lesions were suspected from these histological findings. A similar case that showed IgM, C3 depositions in glomeruli has previously been reported. The possibility of glomerular lesions being induced by mesalazine should be further researched. From the summary of reported cases, a delay of diagnosis of interstitial nephritis induced by mesalazine has resulted in permanent irreversible renal failure. Intensive monitoring of renal function is required when a patient is treated with mesalazine.

View full abstract

We have experienced rare cases of membranoproliferative glomerulonephritis (MPGN)-like nephritis, which was seen in siblings. Both the brothers had asymptomatic hematuria and proteinuria at an age before 10, 7 and 4 years old, respectively. Renal biopsy revealed proliferative glomerulonephritis, resembling MPGN type III. The family history showed that their father and grandfather suffered from end-stage renal disease, suggesting that MPGN seen in the present sibling cases is hereditary. A review of the literature revealed that familial MPGN is rare, that most of the cases have urinary abnormalities at an age of less than 10 years. and that male preponderance is seen in familial MPGN.

View full abstract