ANCA-associated systemic vasculitis in Japan: clinical features and prognostic changes

Background

This study was conducted to standardize treatment and determine patient and renal outcome in Japanese anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis/rapidly progressive glomerulonephritis (AAV/RPGN) patients, because the prognosis of AAV/RPGN patients in Japan had been poor compared with that of other countries.

Methods

The participants in this retrospective cohort study were 824 ANCA-positive RPGN patients, 705 of whom were only myeloperoxidase (MPO)-ANCA positive.

Results

Among the early-years cohort (group?A; cases diagnosed between 1988 and 1998), patients frequently died due to opportunistic infection. Therefore, we recommended a reduced dose of prednisolone (oral prednisolone dose <0.8?mg/kg/day) with or without cyclophosphamide for initial treatment of Japanese RPGN patients. After this recommendation, 1-year survival of the patients improved: 75% in group?A, 79% in group?B (between 1999 and 2002), and 81% in group?C (after 2003). During the entire observation period, average serum creatinine level at the start of treatment decreased, and improvement of 1-year renal survival was also found (72% in group?A, 83% in group?B, and 83% in group?C), while the recurrence rate was significantly increased in group?C (0.05/patient-year in group?A, 0.07/patient-year in group?B, and 0.13/patient-year in group?C).

Conclusions

Oral prednisolone dose <0.8?mg/kg/day with or without cyclophosphamide as an initial treatment could improve patient survival in older Japanese AAV/RPGN patients. However, maintenance treatment avoiding relapse should be established to improve renal outcomes.     

Renal disease progression in autosomal dominant polycystic kidney disease

Background

Autosomal dominant polycystic kidney disease is a lifelong progressive disorder. However, how age, blood pressure, and stage of chronic kidney disease (CKD) affect the rate of kidney function deterioration is not clearly understood.

Methods

In this long-term observational case study up to 13.9?years (median observation period for slope was 3.3?years), serum creatinine was serially measured in 255 mostly adult patients. The glomerular filtration rate was estimated (eGFR) using a modified Modification of Diet in Renal Disease Study method. The total kidney volume (TKV) has been measured in 86 patients at one center since 2006.

Results

As age increased, eGFR declined significantly (P?Conclusion The declining rate of eGFR was relatively constant and did not correlate with age or eGFR after adolescence. eGFR was already low in young adult patients with hypertension. As age increased after adolescence, eGFR declined and TKV increased similarly between normal and high blood pressure groups. eGFR starts to decline in patients with normal eGFR, suggesting that the decline starts earlier than previously thought.     

Hypobilirubinemia Might be a Possible Risk Factor of End-Stage Kidney Disease Independently of Estimated Glomerular Filtration Rate

Background/Aims: The relationship between serum total bilirubin (TB) and estimated glomerular filtration rate (eGFR) is controversial and there is no report on the association between TB and end-stage kidney disease (ESKD). Methods: We examined the cross-sectional association between TB and eGFR and investigated whether TB can predict ESKD with multivariable logistic regression adjusted for age, sex, and baseline eGFR using hospital-based data. Results: The geometric mean TB of patients with eGFR ≥ 90 mL/min/1.73 m(2) (S1), 8960 mL/min/1.73 m(2) (S2), 59-30 mL/min/1.73 m(2) (S3), 29-15 mL/min/1.73 m(2) (S4), and < 15 mL/min/1.73 m(2) (S5 = ESKD) was 0.55 mg/dL, 0.59 mg/dL, 0.56 mg/dL, 0.47 mg/dL, and 0.36 mg/dL (all p<0.0001 except for S1 vs. S3 where p=0.3726), respectively excluding patients with hyperbilirubinemia (TB > 1.24 mg/dL). The odds ratio (95% confidence interval) of incident ESKD for each 0.1 mg/dL increase in TB and hypobilirubinemia defined as TB ≤ 0.34 mg/dL were 0.92 (0.80-1.07) (p=0.2804) and 3.51 (1.56-7.88) (p=0.0023), respectively in patients with baseline eGFR ≥ 15 mL/min/1.73m(2) and 0.59 (0.37-0.95) (p=0.0283) and 6.03 (1.63-22.30) (p=0.0071), respectively in patients with baseline eGFR 29-15 mL/min/1.73m(2). Conclusions: Hypobilirubinemia might be a possible risk factor of ESKD.     

Revision of tumor prosthesis of the knee joint

BACKGROUND: Among 40 patients with primary malignant tumors of the knee joint who underwent reconstruction of the affected limb with tumor prosthesis, revision was required in 7 due to stem breakage or loosening. SUBJECTS AND METHODS: In the 7 cases undergoing revision, conditions and background factors at the time of breakage, the breakage site, time of revision, models of previous and new prostheses, stem diameters before and after revision, details of the revision (blood loss, operative time), and the presence or absence of adjuvant therapy were determined. RESULTS: The replacement site was the distal femur in 5 and proximal tibia in 2. Revision was performed 6?years and 2?months after the previous prosthesis placement on average. The broken prosthesis model was KMFTR in 4 and HMRS and the physio-hinge type in one each. Revision due to loosening was performed in a case requiring replacement with Growing Kotz prosthesis. The model was switched to HMRS in 3, and the stem diameter was changed to 12?mm in 3 KMFTR breakage cases. The mean stem diameters were 11.2 and 10.2?mm in the non-revision and revision groups. The respective resection rates were 36 and 45%. The mean functional evaluation was 70.1% before and 76.2% after revision. CONCLUSION: To reduce the risk of tumor prosthesis breakage, the amount of bone resection should be limited to 30% or less in the affected bone, the stem diameter should be at least 12?mm, and the stem shape should be fitted to the anatomical shape of the femur.     

Supportive techniques and devices for endoscopic submucosal dissection of gastric cancer

The indications for endoscopic treatment have expanded in recent years,and relatively intestinal-type mucosal stomach carcinomas with a low potential for metastasis are now often resected en bloc by endoscopic submucosal dissection(ESD),even if they measure over 20 mm in size.However,ESD requires complex maneuvers,which entails a long operation time,and is often accompanied by complications such as bleeding and perforation.Many technical developments have been implemented to overcome these complications.The scope,cutting device,hemostasis device,and other supportive devices have been improved.However,even with these innovations,ESD remains a potentially complex procedure.One of the major difficulties is poor visualization of the submucosal layer resulting from the poor countertraction afforded during submucosal dissection.Recently,countertraction devices have been developed.In this paper,we introduce countertraction techniques and devices mainly for gastric cancer.     

A case of spontaneous splenic rupture during chemotherapy for B-cell chronic lymphoid leukemia

Spontaneous splenic rupture is a life-threatening disease and an important differential diagnosis of acute abdomen. Early clinical diagnosis and rapid intervention is required to ensure patient survival. Spontaneous splenic rupture may be induced by hematological, inflammatory or infiltrative diseases affecting the spleen. Splenomegaly may also significantly increase the risk of rupture. Other contributory factors include male, adulthood, rapid growth of the spleen and splenic abscess. Here, we present the case of a 69-year-old man who was undergoing chemotherapy for B-cell chronic lymphoid leukemia. He was admitted to our hospital after he suddenly developed persistent upper abdominal pain. Computed tomography and ultrasonography revealed accumulation of free fluid in and around the spleen. He was diagnosed as having spontaneous splenic rupture and an emergency operation was performed. During the operation, we found a massively enlarged spleen with several capsular tears, and performed a splenectomy. The patient made a good recovery. Pathological examination revealed that the spleen was infiltrated by CD20-, CD5- and CD23-positive lymphoid blasts. We encountered a case of spontaneous splenic rupture in a patient receiving chemotherapy for exacerbating B-cell chronic lymphoid leukemia. In a case of abdominal pain of acute onset in patients with hematological disease, spontaneous splenic rupture should be suspected.     

An Overview of Regular Dialysis Treatment in Japan (As of 31 December 2010)

A nationwide statistical survey of 4226 dialysis facilities was conducted at the end of 2010, and 4166 facilities (98.6%) responded. The number of new patients introduced into dialysis was 37 512 in 2010. This number has decreased for two consecutive years since it peaked in 2008. The number of patients who died in 2010 was 28 882, which has been increasing every year. The number of patients undergoing dialysis at the end of 2010 was 298 252, which is an increase of 7591 (2.6%) compared with that at the end of 2009. The number of dialysis patients per million at the end of 2010 was 2329.1. The crude death rate of dialysis patients in 2010 was 9.8%, and has been gradually increasing. The mean age of the new patients introduced into dialysis was 67.8 years and the mean age of the entire dialysis patient population was 66.2 years. Regarding the primary disease of the new patients introduced into dialysis, the percentage of patients with diabetic nephropathy was 43.6%, which is a slight decrease from that in the previous year (44.5%). Patients with diabetic nephropathy as the primary disease accounted for 35.9% of the entire dialysis patient population, which approaches the percentage of patients with chronic glomerulonephritis as the primary disease (36.2%). The percentage of patients who had undergone carpal tunnel release surgery (CTx) was 4.3%, which is a slight decrease from that at the end of 1999 (5.5%). The decrease in the percentage of patients who had undergone CTx was significant among the patients with dialysis durations of 20–24 years (1999, 48.0%; 2010, 23.2%). A total weekly Kt/V attributable to peritoneal dialysis and their residual functional kidney was 1.7 or higher for 59.4% of patients who underwent peritoneal dialysis.     

Late‐type port‐site recurrence of unexpected gallbladder carcinoma 11 years after laparoscopic cholecystectomy

A 55‐year‐old man had laparoscopic cholecystectomy for acute cholecystitis and unexpected gallbladder cancer, followed by a liver bed resection and lymph node dissection. Eleven years later, he had a port‐site recurrence of gallbladder cancer requiring resection; at that time, no other site of recurrence was observed. The patient has survived for 20 months without another recurrence. Although a rare finding, clinicians should be alert to the possibility of such a recurrence even 11 years after complete cure of the primary tumor, particularly in patients who have undergone laparoscopic cholecystectomy for unexpected gallbladder cancer.