[Effect of an educational admission program for patients at the conservative phase of chronic kidney disease (CKD)].

PURPOSE: We have been conducting a 1-week educational admission program for patients at the conservative phase of chronic kidney disease (CKD) since 2006. In this study we evaluated the effect of the program. METHODS: We retrospectively reviewed 469 patients who could be followed for 12 months after a 1-week educational admission program for CKD out of a total of 700 patients who attended the program between October 2006 and April 2012. We compared the rates of decrease in renal function before and after the program. In addition, we divided the patients into two groups of diabetic nephropathy and non-diabetic nephropathy. We compared the rate of decrease in renal function in each group. RESULTS: The rate of decrease in renal function 12 months after discharge was improved compared with that 6 months before admission. (before: 0.316 mL/min/1.73 m2/month; after: 0.001 mL/min/1.73 m2/month.) The rate of decrease in renal function 6 months before admission of the diabetic nephropathy group was 72.3 times faster than that of the non-diabetic nephropathy group. However, the rate of decrease in renal function 12 months after admission was improved in both groups. CONCLUSION: It was revealed that the educational admission program is effective for preserving the renal function on patients at the conservative phase of CKD.

[A case of tuberculous peritonitis in a hemodialysis patient revealed by severe diarrhea and stomachache].

A 53-year-old woman was admitted to our hospital due to abdominal pain, diarrhea, and shunt occlusion caused by dehydration. She had undergone hemodialysis due to diabetic nephropathy over a ten-year period. She was hospitalized again with fever and a persistent high serum CRP level. We started antibiotic administration using cefotiam hexetil hydrochloride because of ascites and peritoneum thickening observed by abdominal computed tomography. Although her symptoms, such as abdominal pain and diarrhea, improved after the administration of antibiotics, the ascites and the peritoneum thickening did not improve. On the fourth hospital day, we attempted ascites aspiration to investigate the etiology of the peritonitis. Cytological examination suggested tuberculous peritonitis because of predominant macrophage cell proliferation, a high level of ADA concentration, and a high level of CA125 of ascites. Although QuantiFERON-tuberculosis (QFT) and the Gaffky scale were negative, we started multidrug therapy (isoniazid + rifampicin + pyrazinamide + ethambutol) on the 20th hospital day. She was finally diagnosed as mycobacterium tuberculous peritonitis based on biopsy of the tissue of the ileum and the results of colonoscopy. Administration of antituberculosis chemotherapy improved abdominal fullness and ascites and the patient was discharged on the 97th hospital day. Moreover Kuno et al. reported that serum soluble interleukin-2 receptor(sIL-2R) and CA-125 levels can be used to monitor the response to anti-tuberculosis treatment. In this case, we use these markers to monitor the response to treatment. We experienced a case of tuberculous peritonitis undergoing hemodialysis. Tuberculosis should be suspected when patients undergoing dialysis have long-term fever of unknown etiology. There are many reports stating that the sensitivity and specificity of QuantiFERON-tuberculosis (QFT) and sputum culture are low in latent tuberculosis infection of dialysis patients. Accordingly it is necessary to diagnose mycobacterium tuberculous peritonitis comprehensively by the clinical symptoms and image analysis.

[Case of lupus nephritis complicated with hemophagocytic syndrome].

A 27-year-old woman was referred to our hospital because of pancytopenia and nephritic syndrome in November, 2008. The findings of physical and laboratory examinations showed systemic lupus erythematosus (SLE). Diffuse proliferative lupus nephritis(group IV-G(A))was confirmed by renal biopsy. After combined therapy with prednisolone, intravenous cyclophosphamide pulse and mizoribine, proteinuria decreased from 13.0 g/day to 2.0 g/day and the serum complement level recovered to the normal level. However, she visited our hospital again for management of bleeding tendency in July 2009. She was diagnosed as hemophagocytic syndrome (HPS), with pancytopenia, high ferritin, high LDH level and hemophagocytosis in the bone marrow. She was treated effectively with steroid pulse therapy, but relapsed with HPS after two weeks. Although her child caught a cold, the case did not show any sign or symptom of infection, such as the common cold. However, we diagnosed her HPS as infection-associated hemophagocytic syndrome (IAHS) because she was not in the active phase of SLE at the onset of hemophagocytosis and the laboratory findings showed elevation of her serum ferritin and LDH. Therefore, we considered that her infectious sign may have been concealed by immunosuppressive therapy with prednisolone for SLE. It is very difficult to distinguish between IAHS and autoimmune-associated hemophagocytic syndrome (AAHS)in autoimmune diseases, but the differential diagnosis is necessary to treat the HPS. Here, we report an important case of HPS complicated with SLE. This case may attract interest particularly in the management of HPS-complicated autoimmune disease. Therefore, we report it with a review of the literature.

[Case of peritubular capillary dominant intravascular large B-cell lymphoma (PTC dominant IVLBCL) successfully treated with chemotherapy].

A 72-year-old woman developed common cold-like symptoms, diarrhea, a staggering gait, and persistent anorexia from the beginning of May 2009. In the middle of May, her general fatigue worsened, and she was transported to our hospital by ambulance. Abdominal CT showed bilateral renal enlargement, and her general condition and renal function rapidly deteriorated. The soluble interleukin-2 receptor (sIL-2R) level was elevated to 5,928 U/mL, and gallium scintigraphy showed a weak uptake in both kidneys. We considered the possibility of malignant lymphoma, and performed a renal biopsy, which showed no glomerular abnormalities, but disclosed the accumulation of large, atypical lymphoid cells with a high N/C ratio and dark chromatin in peritubular capillaries (PTC). On immunohistochemical staining, these atypical cells were found to be CD5(+), CD20 (+/-), CD10(-), CD3(-), and CD7(-), leading to a diagnosis of intravascular large B-cell lymphoma (IVLBCL). Since gallium scintigraphy showed no uptake in other organs, and examination of the cerebrospinal fluid and bone marrow revealed no tumor cells, the patient was considered to have kidney-limited IVLBCL. Chemotherapy was started immediately, which resulted in an improved general condition. Although her renal function deteriorated sufficiently to require dialysis, she was weaned from dialysis. After treatment with chemotherapy, the enlarged kidneys returned to the normal size. Subsequently, she has been receiving chemotherapy intermittently, and has remained free of recurrence. In general, IVLBCL mainly involving the kidney is difficult to diagnose antemortem, and is sometimes found at autopsy. We suggest that bilateral renal enlargement with renal failure of unknown origin should raise the suspicion of malignant lymphoma requiring a prompt renal biopsy. Cases of LBCL in which lymphoma cells fill PTC, as in this patient, have rarely been reported. We believe that this case is extremely valuable in understanding the pathogenesis of intravascular lymphoma invading the kidney; therefore, we report it with a review of the literature.

[Case of fanconi syndrome positive for anti-M2 antibodies revealed by severe hypokalemia and multiple bone fracture].

A 38-year-old female developed pain in the right leg in 2006. In 2007, the diagnosis of femoral head necrosis was made based on MR images, and femoral head prosthetic replacement was performed. In April 2009, she visited a local hospital for low back pain, and was referred to our department due to electrolyte abnormalities on hemanalysis. Since marked hypokalemia (K=2.5 mEq/L), hypophosphatemia, hyperchloric metabolic acidosis, proteinuria, and urinary blood sugar suggested Fanconi syndrome, she was admitted for close examination. Bone survey showed a marked decrease in the amount of bone particularly in the four limbs and fracture at the proximal 1/3 of the left ulnar bone. In the lumbar spine, scoliosis and vertebral deformity were observed. Since impaired P re-absorption and unselected aminoaciduria and osteomalacia were also present, the diagnosis of Fanconi syndrome was made. On admission, ventricular tachycardia developed due to hypokalemia, requiring immediate electrolyte correction. For differentiation from acquired Fanconi syndrome, various examinations were performed. No apparent cause was found except for the positive antimitochondrial antibody-M2 (anti-M2). In this case, no data suggested liver dysfunction, and subsequent liver biopsy also showed no significant pathological findings pointing to PBC. We encountered a patient with Fanconi syndrome positive for anti-M2. This case may attract interest, particularly in the mechanism of nephropathy due to anti-M2, and therefore, this case is reported with a literature review.

Efficacy of low-density lipoprotein apheresis in patients with peripheral arterial occlusive disease undergoing hemodialysis treatment.

BACKGROUND: Low-density lipoprotein (LDL) apheresis is effective in the treatment of peripheral arterial occlusive disease (PAOD). In the present study, we attempted to determine whether LDL apheresis is effective even for PAOD patients undergoing hemodialysis, who tend to be refractory to any treatment, and if so, to determine the mechanism of its efficacy. METHODS: Serum levels of lipids and vascular growth factors, leg symptom, and endothelium-dependent vasodilation were investigated before and after 10 sessions of LDL apheresis in 11 PAOD patients undergoing hemodialysis. RESULTS: Serum levels of total cholesterol, LDL cholesterol, and triglyceride exhibited drastic reduction, which completely disappeared 4 weeks after the final apheresis. Resting leg pain was improved in 6 cases even 4 weeks after final apheresis. Endothelium-dependent vasodilation was significantly increased 4 weeks after final apheresis (1.6 +/- 0.6 to 4.7 +/- 1.0%, p < 0.05). Levels of vascular growth factors, hepatocyte growth factor and vascular endothelial growth factor were not changed during treatment. CONCLUSIONS: These findings suggested that LDL apheresis is effective even in PAOD patients undergoing hemodialysis. Our findings suggest that its mechanisms of efficacy include improvement of vascular endothelial dysfunction, in addition to drastic but acute reduction of lipid levels. Since PAOD patients undergoing hemodialysis tend to be resistant to any treatment and are at high risk for lower-extremity amputation, LDL apheresis could be a useful strategy for treatment of them.

Renal and vascular protective effects of telmisartan in patients with essential hypertension.

It is known that the angiotensin receptor blockers (ARBs) have organ protective effects in patients with heart failure or renal impairment. Several studies have revealed that the ARB telmisartan has an organ protective effect, but there have been few studies directly comparing the effects of telmisartan and calcium antagonists, since most clinical studies on telmisartan have been conducted in treated patients or patients on combination therapy. The present study was conducted to compare the renal and vascular protective effects of telmisartan monotherapy and calcium antagonist monotherapy in untreated hypertensive patients. Forty-three patients with untreated essential hypertension were randomized to receive amlodipine (n=22) or telmisartan (n=21), which were respectively administered at doses of 5 mg and 40 mg once daily in the morning for 24 weeks. The patients were examined before and after treatment to assess changes of renal function, flow-mediated dilation (a parameter of vascular endothelial function), and brachial-ankle pulse wave velocity (baPWV; a parameter of arteriosclerosis). Before treatment, there were no significant differences in these parameters between groups. The decreases of urinary albumin excretion and baPWV, and the increase of flow-mediated dilation were significantly greater in the telmisartan group than the amlodipine group, while the antihypertensive effects were not significantly different between the two groups. In conclusion, these results suggest that telmisartan is more effective at protecting renal function and vascular endothelial function, and at improving arteriosclerosis than the calcium channel blocker in patients with essential hypertension.