Henoch-Schönlein purpura complicated with severe gastrointestinal bleeding.

Bleeding from the gastrointestinal tract is one of the common determinants of morbidity and mortality in the ordinary clinical setting. The gastrointestinal involvement of Henoch-Schönlein purpura (HSP) has often been described as self-limiting, with no long-term morbidity. In this report, we describe our experience with a male HSP patient who presented with abdominal pain, loss of appetite and deteriorated renal function associated with nephrotic syndrome. Despite the use of aggressive immunomodulatory treatments, including corticosteroids and plasmapheresis, he developed lethal gastrointestinal hemorrhage. We believe that the accumulation of more experience with additional cases similar to ours is mandatory for the establishment of optimal management for HSP patients with severe gastrointestinal manifestations.

Hemodialysis without Anticoagulation for a Patient with Chronic Disseminated Intravascular Coagulation.

We report a 68-year-old Japanese man with end-stage renal failure requiring hemodialysis and chronic disseminated intravascular coagulation (DIC) related to thrombosis in an aortic aneurysm. He had undergone graft replacement for the dissection of the ascending and descending thoracic aorta in 1990 and 2002, respectively. Computed tomography disclosed an aneurysm with thrombosis in the residual aorta adjacent to the graft anastomosis. DIC was diagnosed based on elevation of serum fibrinogen degradation products while his activated partial thromboplastin time, prothrombin time and fibrinogen level were normal. In 2008, hemodialysis was initiated for end-stage renal failure. Dialysis was performed without administration of an anticoagulant because his activated clotting time (ACT) was prolonged to 150-180 s. Thereafter, stable hemodialysis continued without clotting in the dialysis circuit until 2013. If monitoring of ACT can be done, hemodialysis without anticoagulation may be a therapeutic option in such patients.

Primary care physicians' own exercise habits influence exercise counseling for patients with chronic kidney disease: a cross-sectional study.

BACKGROUND: The appropriate exercise counseling for chronic kidney disease (CKD) patients is crucial to improve their prognosis. There have been few studies about exercise counseling by primary care physicians for CKD patients. We investigated primary care physicians' exercise counseling practices for CKD patients, and the association of these physicians' own exercise habits with exercise counseling. METHODS: The population of this cross-sectional study was 3310 medical doctors who graduated from Jichi Medical University from 1978 to 2012. The study instrument was a self-administered questionnaire that was mailed in August 2012 to investigate their age class, specialty, workplace, exercise habits, and practices of exercise counseling for CKD. RESULTS: 581 (64.8%) medical doctors practiced the management of CKD among a total of 933 responses. These 581 medical doctors were defined as CKD primary care physicians and their answers were analyzed. CKD primary care physicians' own exercise habits (frequencies and intensities) were as follows: frequencies: daily, 71 (12.1%); ≥ 2-3 times/week, 154 (26.5%); ≥ 1 time/week, 146 (25.1%); and ≤ 1 time/month, 176 (30.2%); intensities: high (≥ 6 Mets), 175 (30.1%); moderate (4-6 Mets), 132 (22.7%); mild (3-4 Mets), 188 (32.3%); very mild (<3 Mets), 47 (8.1%); and none, 37 (6.4%). The CKD primary care physicians' exercise recommendation levels for CKD patients were as follows: high, 31 (5.3%); moderate, 176 (29.7%); low, 256 (44.0%); and none, 92 (15.8%). The CKD primary care physicians' exercise recommendations for CKD patients were significantly related to their own exercise frequency (p < 0.001), but they were not related to their age, specialty, workplace, or exercise intensity. CONCLUSIONS: CKD primary care physicians' exercise recommendation level for CKD patients was limited. In addition, CKD primary care physicians' own exercise habits influenced the exercise counseling for CKD patients. The establishment of guidelines for exercise by CKD patients and their dissemination among primary care physicians are needed.(University Hospital Medical Information Network Clinical Trial Registry. number, UMIN000011803. Registration date, Sep/19/2013).

Purpura with ulcerative skin lesions and mixed cryoglobulinemia in a quiescent hepatitis B virus carrier.

Mixed cryoglobulinemia is occasionally seen in patients with hepatitis B virus (HBV) infection. This report presents the case of a quiescent HBV carrier who had type II mixed cryoglobulinemia, protracted purpura, ulcerative skin lesions and advanced chronic kidney disease. The cutaneous manifestations of the patient improved along with a decrease in the serum cryoglobulin and HBV-deoxyribonucleic acid levels following the initiation of oral entecavir in combination with plasmapheresis. However, the patient ultimately required prednisolone due to the limited benefits of these treatments. We also discuss various concerns regarding steroid treatment in patients with mixed cryoglobulinemia complicated by HBV infection.

Medication-prescribing patterns of primary care physicians in chronic kidney disease.

BACKGROUND: We investigated the medication-prescribing patterns of primary care physicians in chronic kidney disease (CKD). SUBJECTS AND METHODS: This cross-sectional study included 3,310 medical doctors who graduated from Jichi Medical University. The study instrument was a self-administered questionnaire to investigate their age group, specialty, workplace, existence of a dialysis center at workplace, and their prescription frequencies (high, moderate, low, very low) of the following agents--calcium (Ca) inhibitors, angiotensin-converting enzyme inhibitors (ACEIs), angiotensin II receptor antagonist (ARBs), statins, anti-platelet agents, erythropoietin (Epo), AST-120, vitamin D, and sodium hydrogen carbonate (NaHCO(3)). RESULTS: From a total of 933 responses, 547 (61.0 %) medical doctors prescribed medication for CKD. The prescription frequencies of Ca inhibitors, ACEIs, and ARBs were high (>90 %, high + moderate), those of statins, anti-platelet agents, Epo, and AST-120 were moderate (90-50 %, high + moderate), and those of vitamin D and NaHCO(3) were low (<50 %, high + moderate). The primary care physician's specialty was significantly associated with their prescription frequency of Ca inhibitors (p < 0.01). Their workplace was significantly associated with their prescription frequency of ACEIs (p < 0.01), ARBs (p < 0.01), Epo (p < 0.01) and vitamin D (p < 0.01). The existence of a dialysis center at their workplace was significantly associated with their prescription frequency of Epo (p < 0.01), vitamin D (p < 0.01) and NaHCO(3) (p < 0.01). Their age was not associated with their prescription frequency of any agents. CONCLUSION: Antihypertensives were highly prescribed, and vitamin D and NaHCO(3) were less prescribed by primary care physicians for CKD. There were certain associations between the prescribing patterns of primary care physicians for CKD and their specialty, workplace and the existence of a dialysis center at their workplace.

Acute post-streptococcal glomerulonephritis with acute kidney injury in nephrotic syndrome with the glomerular deposition of nephritis-associated plasmin receptor antigen.

We herein report the case of a 17-year-old man who developed an increased plasma creatinine level (11.1 mg/dL) and oliguria with massive proteinuria (27.3 g/day) four weeks after an abraded wound to his right knee. The histology of the renal biopsy specimens showed diffuse endocapillary proliferative glomerulonephritis with the deposition of nephritis-associated plasmin receptor in the glomerulus. A case of acute kidney injury due to nephrotic syndrome caused by acute post-streptococcal glomerulonephritis was diagnosed. His renal function and proteinuria were improved with supportive care, including hemodialysis, without the administration of immunosuppressive agents.

Acute kidney injury in a patient with nephrotic syndrome due to focal segmental glomerular nephritis induced by a single oral administration of high-dose bisphosphonate (minodronate).

We herein report the case of a 75-year-old man who developed an increased serum creatinine level (4.93 mg/dL) and oliguria with massive proteinuria (7.14 g/day) on the second day after a single oral administration of high-dose (56 mg) minodronate. The histology of a renal biopsy showed one area of glomerular sclerosis among 20 glomeruli with global foot process effacement of podocytes and mild infiltration of lymphocytes and eosinophils into the interstitial space. Acute kidney injury in nephrotic syndrome due to focal segmental glomerular sclerosis induced by minodronate was diagnosed. Following cessation of minodronate without the administration of immunosuppressive agents, the patient's renal function and proteinuria markedly improved.

A case of IgG4-related tubulointerstitial nephritis with left hydronephrosis after a remission of urinary tract tuberculosis.

IgG4-related systemic disease encompasses multi-organ disorders, including tubulointerstitial nephritis. This disease is accompanied by a high serum IgG4 concentration and IgG4-positive plasma cell infiltration. We herein describe a 63-year-old woman with renal failure and dryness of the eyes and mouth, who had been treated with antituberculosis agents for urinary tract tuberculosis. She had a negative finding for a PCR analysis for Mycobacterium tuberculosis, a positive QuantiFERON-TB test, high serum IgG4 concentrations (2,660 mg/dl), and low serum IgM and IgA concentrations (34 and 82 mg/dl, respectively). Imaging tests revealed swelling in the submandibular glands, pancreas, and right kidney. A renal biopsy showed IgG4-positive plasma cell infiltration in the interstitium and tubular atrophy. This case was diagnosed as IgG4-related systemic disease. Corticosteroid therapy improved renal failure and swelling in the submandibular glands, pancreas, and right kidney. The case suggests that an abnormal reaction to tuberculosis may be associated with a predominance of type-2 helper T-cell immunity, thus resulting in IgG4-related systemic disease.

De novo postallogeneic hematopoietic stem cell transplant membranous nephropathy.

We report membranous nephropathy in a 61-year-old man after allogeneic hematopoietic stem cell transplant without chronic graft-versus-host disease. A diagnosis of acute myeloid leukemia was made, and the patient received hematopoietic stem cell transplants, twice, from different donors. The first donor was his brother and the second donor was an unrelated man. Human leukocyte antigens between donors and recipient were fully matched. His clinical course was stable without acute or chronic graft-versus-host disease or relapse of acute myeloid leukemia with tacrolimus after the second hematopoietic stem cell transplant. Six months after the second hematopoietic stem cell transplant, tacrolimus was decreased gradually and discontinued because of tacrolimus-induced liver dysfunction. Three months after discontinuing the tacrolimus, the patient developed edema in his leg. The results of a blood analysis showed that plasma albumin level was 21 g/L and plasma total cholesterol level was 11.5 mmol/L, while results from a urinalysis showed proteinuria of 5.6 g/d without hematuria. No abnormalities in the skin, mucosal tissues, and other organs suggestive of chronic graft-versus-host disease were seen. A renal biopsy was done to investigate the cause, which revealed renal disease. Electron microscopic analysis showed dense deposits in the subepithelial region in all glomeruli. Immunofluorescence analysis showed the deposition of IgG4 and C3c in the subepithelial space of all glomeruli. Membranous nephropathy was diagnosed. He then was administered prednisolone at a dosage of 45 mg/d (0.7 mg/kg/d). After prednisolone treatment, urine protein and hypoalbuminemia were markedly improved, and his leg edema disappeared. These results suggest that this membranous nephropathy may have been de novo membranous nephropathy after hematopoietic stem cell transplant because it developed after hematopoietic stem cell transplants without chronic graft-versus-host disease.

Rituximab Treatment for PR3-ANCA-Positive Membranoproliferative Glomerulonephritis Associated with Adult-Onset Periodic Fever Syndrome.

We report the case of a 36-year-old Japanese woman with nephrotic syndrome due to membranoproliferative glomerulonephritis (MPGN) Type I diagnosed after a 5-year history of periodic fever syndrome (PFS). Hypocomplementemia and elevation of anti-proteinase 3 anti-neutrophil cytoplasmic autoantibody (PR3-ANCA) were observed. HIV, and hepatitis B and C serology were negative. Nephrotic syndrome and periodic fever did not respond to oral steroid and intravenous steroid pulse therapies combined with cyclosporine, dipyridamole, warfarin and losartan. We tried immunotherapy using rituximab, a human-mouse chimeric monoclonal antibody directed against the CD20 antigen on mature B cells. This therapeutic approach led to improvement of renal function and remission of nephrotic syndrome and hypocomplementemia. However, it did not have a beneficial effect on periodic fever. Suspecting adult-onset hereditary PFS, we analyzed her genetic alteration of MEFV and TNFRSF1A genes. A rare genotype in intron 6 of TNFRSF1A was revealed. The etiological relationship between periodic fever and MPGN is discussed. Rituximab is a hopeful choice of induction therapy for refractory MPGN.

Minimal change nephrotic syndrome complicated with malignant ascites in a patient with type II diabetes.

A large number of renal biopsy studies have shown the concurrent presence of non-diabetic renal disease in diabetics. This report describes one such diabetic female patient with nephrotic syndrome due to minimal change glomerular disease who was successfully treated with prednisolone. Despite the remission of her nephrotic syndrome, she had gradual development of malignant ascites, which was finally interpreted to be linked to primary peritoneal carcinoma. It is necessary to bear in mind that malignancies may not only be the underlying etiology for paraneoplastic glomerular injuries, but also can be an independent pathogenic process, regardless of their nephrotic status during the overall management of the patients with ascites.

Successful treatment of a mesangial proliferative glomerulonephritis with interstitial nephritis associated with Castleman's disease by an anti-interleukin-6 receptor antibody (tocilizumab).

We report a case of mesangial proliferative glomerulonephritis with interstitial nephritis associated with multicentric Castleman's disease (MCD) successfully treated with an anti-interleukin-6 receptor antibody (tocilizumab). This mesangial proliferative glomerulonephritis with interstitial nephritis was resistant to methylprednisolone treatment; however, it was markedly improved with tocilizumab, which was administered intravenously at a dose of 8 mg/kg every 2 weeks. These results suggest that tocilizumab is effective for the treatment of mesangial proliferative glomerulonephritis with interstitial nephritis associated with MCD.

Churg-Strauss syndrome presenting with acute renal insufficiency accompanied by eosinophilic tubulointerstitial nephritis.

We encountered an unusual and rare case of 59-year-old woman with Churg-Strauss syndrome (CSS) showing myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)-related acute renal insufficiency accompanied by eosinophilic tubulointerstitial nephritis. To date, reports in English of CSS presenting with rapidly progressive/acute renal insufficiency and biopsy-proven renal lesions have been uncommon. Here, we discuss this unusual case and review the previously reported CSS cases. The complication of eosinophilic tubulointerstitial nephritis in CSS cases with acute renal insufficiency might be higher than generally thought. Furthermore, the presence of eosinophilic infiltration and eosinophilic tubulointerstitial nephritis might be associated with the good renal outcome in CSS patients.

ANCA-associated crescentic glomerulonephritis with immune complex deposits.

BACKGROUND: Several authors have reported cases of anti-neutrophil cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis (CGN) with definite immune complex (IC) deposits, however, the clinical and pathological significance of IC deposits in patients with ANCA-associated CGN remains unclear. METHODS: Renal biopsy specimens from 28 patients with a diagnosis of CGN and positivity for anti-myeloperoxidase (MPO)-ANCA were retrospectively evaluated. Clinical data were compared between patients with IC deposits (Group A) and patients without IC deposits (Group B). RESULTS: In 12 patients (43%; Group A), IC deposits were detected in the mesangium and/or along the glomerular capillary walls, while typical pauci-immune CGN without IC deposits was found in 16 patients (57%; Group B). Compared with Group B, Group A had lower levels of MPO-ANCA (171 ± 156 vs. 352 ± 299 EU) and C-reactive protein (CRP) (0.63 ± 1.04 vs. 4.45 ± 4.00 mg/dl), as well as less pulmonary involvement (8.3% vs. 56.3%) at diagnosis. However, Group A had significantly heavier proteinuria (2.46 ± 1.67 vs. 0.76 ± 0.52 g/d). Group A was classified into three subgroups: Group A1 with mesangial IgA and C3 deposits, A2 with mesangial IgG and C3 deposits, and A3 with IgG and C3 deposits mainly in the capillary walls. CONCLUSIONS: ANCA-associated CGN causes two types of renal involvement, which are the pauci-immune type without IC deposits and the IC deposition type that involves three patterns of IC deposition in the glomeruli. The reason why IC deposits are associated with renal-limited vasculitis and not systemic vasculitis remains unclear.

Is there a link between diabetic glomerular injury and crescent formation? A case report and literature review.

Glomerular crescents are most commonly associated with rapidly progressive crescentic glomerulonephritis; however, they also develop in response to a wide range of primary and secondary glomerular injuries. Since various kind of glomerulopathies occasionally overlay diabetic glomerular injuries, the presence of crescents in renal biopsy specimens of diabetics may have stimulated a search for etiologies other than diabetes. In this report, we describe an unusual case of diabetic glomerulosclerosis with peculiar extracapillary proliferation. Although such a relationship has so far been ignored in most of the literature, the etiological linkage between diabetic glomerulosclerosis and the development of crescents may not be exceptional. We have reviewed the previous literature and herein discuss the pathological implications of the development of crescents in patients with diabetic glomerulosclerosis. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/3950457896920255.

A case of diffuse endocapillary proliferative glomerulonephritis associated with polymyalgia rheumatica.

A 70-year-old man complained of muscle pain in his neck, shoulders and pelvic girdle. Proteinuria and hematuria subsequently developed. Blood analysis showed increased acute phase reactants. The histology of renal biopsy showed diffuse endocapillary proliferative glomerulonephritis. There were no signs of autoimmune diseases, malignancies and bacterial or viral infections. His extrarenal symptoms and the results of blood analysis fulfilled three different criteria of polymyalgia rheumatica (PMR). Therefore, diffuse endocapillary proliferative glomerulonephritis associated with PMR was diagnosed. After low-dose prednisolone (10 mg/day) treatment, the muscle pain disappeared, acute phase reactants decreased and hematuria and proteinuria improved. The renal complication of PMR is rare but important to be considered early in the right clinical context.

Successful treatment with tolvaptan to control blood volume and hyponatremia in a chronic kidney disease patient.

We report a case of successful treatment with tolvaptan (15 mg/day) in a 73-year-old female patient with chronic kidney disease (CKD) stage 5 due to diabetic nephropathy and renal sclerosis for volume control and loop diuretic-induced hyponatremia. Her creatinine clearance has remained at 7-10 ml/min for the last 6 months. She was treated by dietary and drug therapy, namely, antihypertensives (nifedipine: 40 mg/day, olmesartan: 20 mg/day) and loop diuretics (azosemide: 40-120 mg/day), for CKD and concomitant diseases of hypertension and diabetic mellitus. She developed loop diuretic-induced hyponatremia (120 mmol/l) by increased sodium excretion, but the diuretic was required for the control of volume overload. Hence, azosemide was suspended and tolvaptan (15 mg/day) was administered. After tolvaptan treatment, the plasma sodium level gradually increased to a normal level (135-140 mmol/l) and volume overload was improved. Urine volume was maintained at about 1000 ml/day with low sodium excretion (<40 mmol/day) and increased free water clearance. These results suggest that tolvaptan may be effective for volume control and diuretic-induced hyponatremia in CKD patients.

Membranous nephropathy in an HIV-positive patient complicated with hepatitis B virus infection.

In ordinary settings, human immunodeficiency virus (HIV)-associated nephropathy should be considered when HIV infection is associated with heavy proteinuria. On the other hand, hepatitis B virus (HBV) may also play a role in the development of glomerular injury among patients with HIV infection, since HIV and HBV infections commonly occur together due to shared modes of transmission. We present here a case of nephrotic syndrome in an HIV-positive patient complicated with HBV infection. A renal biopsy revealed sparse granular deposits of immunoglobulin G in the subepithelial region, consistent with membranous nephropathy (MN) stage I. Moreover, immunostaining exhibited weak anti-hepatitis B core activity within glomeruli. These results led us to consider that HBV-associated MN might play a role in the development of nephrotic syndrome. Although anti-viral treatment for patients with HBV-associated MN has been suggested to be clinically effective, the use of two anti-HIV agents (tenofovir and emtricitabine), both of which have anti-HBV activities, was not effective for the patient's nephrotic syndrome, despite obtaining a decrease in the serum HBV-DNA levels. A lack of prospective data suggests that many decisions on the treatment of glomerulopathies with HIV infections are potentially empirical. Obviously, further studies and accumulated clinical experience are required to better determine the pathogenesis and management of HBV-associated MN among patients with HIV infections.