Hong Kong Society of Clinical Blood Management recommendations for implementation of patient blood management.

Patient blood management (PBM) is a patient-centred, multidisciplinary approach to optimise red cell mass, minimise blood loss, and manage tolerance to anaemia in an effort to improve patient outcomes. Well-implemented PBM improves patient outcomes and reduces demand for blood products. The multidisciplinary approach of PBM can often allow patients to avoid blood transfusions, which are associated with less favourable clinical outcomes. In Hong Kong, there has been increasing demand for blood in the ageing population, and there are simultaneous blood safety and donor issues that are adversely affecting the blood supply. To address these challenges, the Hong Kong Society of Clinical Blood Management recommends implementation of a PBM programme in Hong Kong, including strategies such as optimising red blood cell mass, improving anaemia management, minimising blood loss, and rationalising the use of blood and blood products.

Exploring Muscle Synergies for Performance Enhancement and Learning in Myoelectric Control Maps.

This work proposes two myoelectric control maps based on a DoF-wise synergy algorithm, inspired by human motor control studies. One map, called intuitive, matches control outputs with body movement directions. The second one, named non-intuitive, takes advantage of different synergies contribution to each DoF, without specific correlation to body movement directions. The effectiveness and learning process for the two maps is evaluated through performance metrics in ten able-bodied individuals. The analysis was conducted using a 2-DoFs center-reach-out task and a survey. Results showed equivalent performance and perception for both mappings. However, learning is only visible in subjects that performed better in non-intuitive mapping, that required some familiarization to then exploit its features. Most of the myoelectric control designs use intuitive mappings. Nevertheless, non-intuitive mapping could provide more design flexibility, which can be especially interesting for patients with motor disabilities.

Ten-year review of disease pattern from percutaneous renal biopsy: an experience from a paediatric tertiary renal centre in Hong Kong.

OBJECTIVE: To study the childhood renal disease pattern based on the renal biopsy histology in a local paediatric tertiary renal centre. DESIGN: Retrospective study. SETTING: Department of Paediatrics and Adolescent Medicine, Princess Margaret Hospital, Hong Kong. PATIENTS: All patients who underwent real-time ultrasound-guided closed renal biopsy from 1 April 1997 to 31 March 2007 were included. RESULTS: A total of 209 renal biopsies were performed, 162 on native kidneys and 47 on grafts. In the native group, major indications were renal manifestations secondary to systemic diseases (34%), followed by idiopathic nephrotic syndrome (28%) and haematuria (27%). In 94% the histopathology revealed glomerular diseases. Among the primary glomerular diseases, thin glomerular basement membrane disease, immunoglobulin A nephropathy, minimal change disease, and focal segmental glomerulosclerosis accounted for most. In all, 37% of patients with steroid-resistant nephrotic syndrome had focal segmental glomerulosclerosis and its relative incidence was increased when compared to previous studies. Minimal change disease and minimal change disease with mesangial immunoglobulin M deposits accounted for the majority of steroid dependent and frequent relapsers. Among patients with isolated microscopic haematuria, 73% had thin glomerular basement membrane disease, while patients with concomitant haematuria and proteinuria had a wide variety of pathology. In the kidney graft group, acute graft dysfunction was due to acute rejection in 38% of the patients, followed by calcineurin inhibitor toxicity in 14%. Chronic allograft nephropathy caused chronic allograft dysfunction in the majority of cases. Post-transplant proteinuria was caused by recurrence of the primary renal disease in all of our patients. CONCLUSION: This study provides updated epidemiological information for childhood renal disease and a change in the pattern of disease was observed.

Majocchi's granuloma and posttransplant lymphoproliferative disease in a renal transplant recipient.

Renal transplant recipients are predisposed to infection and malignancy because of underlying long-term immunosuppressive therapy. In this case report, a renal transplant patient with coexisting Trichophyton rubrum granuloma (Majocchi's granuloma) and posttransplant lymphoproliferative disease (PTLD) is presented, showing the undesirable effects of heavy immunosuppression. Majocchi's granuloma was probably associated with PTLD as a reflection of overimmunosuppression.

Nasopharyngeal carcinoma---time lapse before diagnosis and treatment.

This is a descriptive study of 168 patients with nasopharyngeal carcinoma who were referred to public oncology departments for primary treatment between July and September 1996. The mean duration from the onset of the symptoms to histological diagnosis was 5.0 months; the duration ranged from 6.1 months (for patients presenting with nasal symptoms) to 1.8 months (for those with cranial nerve dysfunction). The mean period between the onset of symptoms and the seeking of medical advice was 2.9months. For 54% of the patients, there was a further delay of up to 2.4 months between the initial medical consultation and referral to the appropriate specialist. The majority (84%) of patients attended public institutions for histological confirmation. The mean total time taken from the onset of symptoms to the commencement of radiotherapy was 6.5 months (range, 1.3-74.0 months)---45% of the delay was attributed to the patient, 20% to initial consultations, 14% to diagnostic arrangement, and 21% to preparation for radiotherapy. Concerted efforts are needed to minimise further the time between the onset of symptoms and treatment. A substantial reduction in this delay can be achieved if both public and primary care doctors were made more aware of the significance of relevant symptoms.

Basaloid-squamous carcinoma of the nasal cavity.

Basaloid-squamous carcinoma is a recently characterized, aggressive tumour occurring in the hypopharynx, larynx and base of tongue. We report a unique case involving the nasal cavity of a patient with a history of radiation therapy for nasopharyngeal carcinoma, raising the possibility that basaloid-squamous carcinoma can be radiation-associated.

Rhodococcus lung abscess complicating kidney transplantation: successful management by combination antibiotic therapy.

In this report, a renal transplant recipient with Rhodococcus lung abscess is described. A high clinical suspicion and appropriate combination antibiotic therapy obviated the need for surgical intervention and was associated with a good clinical outcome. The optimal regimen of combination antibiotic therapy is discussed.

A pilot study on tacrolimus treatment in membranous or quiescent lupus nephritis with proteinuria resistant to angiotensin inhibition or blockade.

Persistent proteinuria in patients with quiescent lupus can result from membranous lupus nephritis and/or glomerular scarring following previous flares. This pilot study examined the effects of tacrolimus over two years in six patients with membranous/inactive lupus nephritis and persistent proteinuria despite angiotensin inhibition/blockade. Tacrolimus treatment reduced proteinuria and increased serum albumin (time effect, P = 0.047 and 0.032 respectively). Compared with baseline levels, proteinuria improved by more than 50% in five patients (83.3%) and hypoalbuminaemia was corrected in four patients. The efficacy was most prominent in four patients with biopsy-proven membranous lupus nephritis, whose protienuria improved by over 80%. One patient developed biopsy-proven chronic nephrotoxicity after 10 months of tacrolimus treatment, despite non-excessive blood levels. These data suggest that tacrolimus is an effective treatment for proteinuria due to membranous lupus nephritis, but should probably be reserved for patients who are refractory to other non-nephrotoxic treatments, in view of the potential risk of subclinical nephrotoxicity.

Quality of life comparison between corticosteroid- and-mycofenolate mofetil and corticosteroid- and-oral cyclophosphamide in the treatment of severe lupus nephritis.

There is accumulating evidence that mycophenolate mofetil (MMF), when combined with corticosteroid, is an effective induction treatment for severe proliferative lupus nephritis and is associated with fewer adverse effects compared to cyclophosphamide (CTX), but the quality of life (QOL) associated with these regimens as perceived by the patient has not been compared. This study included patients who had experienced both treatment regimens, for distinct episodes of diffuse proliferative lupus nephritis. QOL parameters during the first six months of each treatment were assessed through SF36 and WHOQOL questionnaires. Twelve patients and 24 episodes of severe lupus nephritis were studied. CTX-treated and MMF-treated episodes showed comparable baseline characteristics and response rate, with complete remission occurring in 83.3%. MMF treatment was associated with higher numerical scores for all domains across both QOL instruments than CTX. MMF treatment was associated with significantly less fatigue, less impediment of physical and social functioning, and better psychological well being compared to CTX. When each patient served as her/his own control, most patients ascribed higher QOL domain scores to the MMF-treated episode. Seventy-five percent of patients found MMF treatment more acceptable and preferred when compared with CTX, and the complications that most concerned them included Cushingoid features, alopecia, menstrual disturbance and infections. These data showed that MMF-based induction immunosuppression for severe lupus nephritis was associated with better QOL than CTX as perceived by patients, which was most likely attributed to the reduced side-effects during MMF treatment.

Angiotensin inhibition or blockade for the treatment of patients with quiescent lupus nephritis and persistent proteinuria.

Angiotensin-converting enzyme inhibitor (ACEI) or angiotensin II receptor blocker (ARB) reduces proteinuria and the rate of renal function deterioration in diabetic nephropathy and other glomerular diseases, but its role in quiescent lupus nephritis has not been established. We conducted a retrospective study to investigate the effects of ACEI/ARB on proteinuria and renal function in patients with persistent proteinuria (>1 g/day) despite resolution of acute lupus nephritis following immunosuppressive treatment. Fourteen out of 92 patients were included. The duration of treatment with ACEI/ARB was 52.1 +/- 35.7 months. The levels of proteinuria, serum albumin, serum creatinine, systolic and diastolic blood pressure were 1.10 to 6.90 g/day, 35.8 +/- 3.6 g/L, 102.54 +/- 34.48 micromol/L, 137.6 +/- 10.9 and 81.9 +/- 9.2 mmHg at baseline. Proteinuria and serum albumin showed significant sustained improvements after 6 and 24 months of treatment. Comparison of slopes for serial proteinuria, albumin and reciprocal of serum creatinine before and after treatment showed significant improvements in six (43%), eight (57%) and two patients, respectively. At last follow-up proteinuria remained significantly lower (0.36 g/day, P = 0.043) and albumin higher (41.3 +/- 2.2 g/L, P = 0.023). Eleven (78.6%) patients had proteinuria improved by >50%, and five had insignificant proteinuria at last follow-up. Systolic blood pressure was significantly reduced from 6 months onwards, but this did not correlate with proteinuria reduction. Diastolic blood pressure, serum creatinine, creatinine clearance, anti-dsDNA, C3 and haemoglobin were not altered. We conclude that ACEI/ARB effectively reduces proteinuria and improves serum albumin in patients with persistent proteinuria despite quiescent lupus nephritis.

Long-term outcome of patients with diffuse proliferative lupus nephritis treated with prednisolone and oral cyclophosphamide followed by azathioprine.

The short-term outcome of patients with diffuse proliferative lupus nephritis (DPLN) has improved with advances in immunosuppressive treatment. However, the impact of different immunosuppressive regimens on long-term renal function remains to be defined. This prospective cohort study examined the long-term renal function and disease relapse in adults with biopsy-proven DPLN, significant proteinuria, and hypoalbuminemia, who had been treated with sequential immunosuppression comprising prednisolone and oral cyclophosphamide as induction followed by low-dose prednisolone and azathioprine as maintenance. Sixty-six patients with 68 episodes of DPLN were included, with follow-up of 91.7 +/- 36.7 months. 82.4% achieved complete remission and 39.1% relapsed during follow-up. Patients in partial remission were at higher risk of relapse compared with those in complete remission (hazard ratio 6.2, P < 0.001). Serum creatinine remained stable over time (P = 0.931), while creatinine clearance showed a significant increase with time after treatment (P = 0.032). Three (4.4%) patients had doubling of baseline creatinine, but none reached end-stage renal failure or died. Univariate and mixed model analyses showed that the evolution of long-term renal function was significantly influenced by the chronicity score and creatinine clearance at baseline, and by the renal function at one year after treatment. These data demonstrate the efficacy of sequential immunosuppression in preserving renal function in most Chinese subjects with DPLN. The results also indicate that irreversible renal scarring (as reflected by baseline chronicity score), renal reserve (as reflected by renal function at baseline and one year), and an induction regimen that is effective in preserving the nephron mass are critical determinants of long-term renal outcome.

Reactivation of systemic lupus erythematosus in a dialysis patient after tuberculous peritonitis.

The disease activity of patients suffering from lupus nephritis usually becomes quiescent after the onset of end stage renal failure. Reactivation of lupus activity, especially after a long period of dialysis, is uncommon. Factors that might trigger off lupus reactivation after dialysis have not been well defined. We report a case of a 43-year-old Chinese woman on long-term peritoneal dialysis, who developed lupus reactivation with cerebral involvement 2 weeks after she was diagnosed to have tuberculous peritonitis. The close temporal relationship between the tuberculous peritonitis and the lupus reactivation raise the possibility that the tuberculous infection might have triggered off the lupus reactivation.

Use of prophylactic lamivudine and mycophenolate mofetil in renal transplant recipients with chronic hepatitis B infection.

Chronic HBsAg carriers are known to have a higher risk of hepatitis-related mortality and morbidity when undergoing kidney transplantation. Immunosuppressants might flare up the infection that could be fulminating. Lamivudine and mycophenolate mofetil (MMF) have been shown to be effective in inhibiting replication of hepatitis B virus (HBV). With these two drugs, hepatitis related adverse outcome might be preventable when these patients are being transplanted. Four Chinese adolescents with chronic HBV infection were transplanted in our Department from 1999 to 2001. Immunosuppresants included prednisolone, cyclosporin A and MMF; azathioprine was not used for its potentially liver toxic effect. Prophylactic lamivudine 3 mg/kg and maximum 100 mg daily was given just before transplantation and was continued afterwards. HBV status and liver enzymes were monitored serially. Patients were followed up for 26.0 +/- 10.3 (11-34) months post-transplant and no mortality was reported. All grafts were functioning and no rejection was noted. MMF and lamivudine were well tolerated. Alanine transaminase was only transiently elevated in the first 2 months post-transplant in all patients and became normal afterwards. The patients were clinically well and liver function was normal at the last follow-up. However, HBV DNA became positive in three patients after the transplantation. YMDD mutant HBV was negative in one patient and undeterminable in the other three due to low virus load. In summary, with prophylactic lamivudine and MMF, short-term follow-up showed that renal transplant might be feasible and safe in chronic HBV carriers.

Clear cell rhabdomyosarcoma of the nasal cavity and paranasal sinuses.

This report describes two cases of alveolar rhabdomyosarcoma of the nasal cavity with unusual histological appearances mimicking clear cell carcinoma. The closely packed tumour cells were polygonal and arranged in sheets and packets. They had an appreciable amount of clear cytoplasm due to accumulation of glycogen. The diagnosis of rhabdomyosarcoma was confirmed by positive staining for desmin and myoglobin. Rhabdomyosarcoma should be included in the differential diagnosis of nasal clear cell tumours, particularly in young adults. A correct diagnosis is important, because chemotherapy is indicated even for apparently localized disease.