Two related Chinese Fabry disease patients with a p.N215S pathological variant who presented with nephropathy.

Fabry disease is an X-linked lysosomal storage disease resulting from a mutation in the GLA gene that encodes α-galactosidase A. The p.N215S (c.644A > G [p.Asn215Ser]) genotype is the most common later-onset variant reported in individuals of European or North American descent. It is usually referred to as a cardiac variant, although manifestations in other organ systems have been observed. In this report, we describe a nephropathy presentation in two related Chinese Fabry disease patients with p.N215S.

Long-term outcomes of living kidney donors: a single centre experience of 29 years.

AIM: Living kidney donation provides the best source of kidney graft. The mortality and morbidity rates are small but the long-term effects have not been studied. This is a report on our 29-year experience of living kidney donation. METHODS: All living donors were arranged to have follow-ups. Defaulters were traced via a territory-wide computer system. RESULTS: A total of 149 living kidney donor operations were performed. 136/149 records were available. 41 defaulted follow-up. One donor died of multiple myeloma. The male to female ratio was 1.00 to 1.52. Mean age at donation was 33.94±9.66 years. Mean follow-up duration was 160.39±87.96 months. Hypertension was diagnosed in 27 donors (19.9%). 22 donors (17.3%) had stage 3 chronic kidney disease (CKD). Glomerular filtration rate (GFR) dropped from 90.95±15.62 mL/min per 1.73 m2 at time 0 to 66.29±12.06 mL/min per 1.73 m2 at 2 years. GFR improved subsequently and remained stable for 25 years. Age at donation was associated with hypertension (HT) in univariate and multivariate analyses. HT was not associated with sex or GFRs over time. Using binary logistic regression, age at donation was associated with the development of stage 3 CKD and GFR before donation was associated with lower CKD risk. In multivariate analysis, only age at donation was associated with CKD. Other co-morbidities included: hyperlipidaemia 16/136, diabetes mellitus 6/136, cardiovascular event 1/136, stroke 1/136 and cancer 5/136. CONCLUSIONS: Living kidney donors had reductions in GFR post uninephrectomy with subsequent improvement. A significant proportion developed HT and stage 3 CKD. Age at donation was a strong determinant of development of HT and stage 3 CKD.

Renal registry and peritoneal dialysis management: the Hong Kong perspective.

The Hong Kong Renal Registry (HKRR) is an electronic paperless registry that services as database for patients on various renal replacement therapies in the territory. The database consists of demographic data, dialysis and transplant treatments, complications, and inquiries and reports. The HKRR can be helpful for individual patient's management, for renal center management, and for territory-wide management.

A prospective study of the efficacy of local application of gentamicin versus mupirocin in the prevention of peritoneal dialysis catheter-related infections.

BACKGROUND: Peritoneal dialysis (PD)-related infections are the major cause of technique failure. Exit-site infections (ESI) can be prevented by local application of antibiotics. Mupirocin (M) is the most extensively studied drug for this application. Long-term use can result in the development of resistance. Gentamicin (G) is an attractive alternative, with both gram-positive and gram-negative activities. We studied the comparative efficacy of G cream versus M ointment in the prevention of PD-related infections in a Chinese cohort. METHODS: This was a prospective study of adult PD patients of the Princess Margaret Hospital, Hong Kong. Patients were excluded if they had active infection, recent ESI or peritontiis, history of allergy to either drug, or were unable to apply the drug or give consent. Patients were taught to apply the drug daily to the exit site after routine exitsite care. Records were tracked prospectively during hospital admissions and clinic follow-ups. RESULTS: 95 patients were recruited; 14 discontinued the study. The ESI rates were 0.38 and 0.20 episodes/patient-year for the G group and the M group respectively (p = 0.36). Gram-positive ESI rates were 0.18 and 0 episodes/patient-year for the G group and the M group respectively. Gram-negative ESI rates were 0.20 episodes/patient-year for both groups (p = 0.62). The overall peritonitis rates were similar in the two groups (p = 0.91). DISCUSSION: In addition to good perioperative care and strict exit-site care, local antibiotic application can prevent ESI. Mupirocin has been extensively studied and shown to be effective. Similar if not superior effects of G cream have been demonstrated. In this study, neither antibiotic gave significantly better results in the prevention of either ESI or peritonitis. CONCLUSIONS: Both gentamicin and mupirocin were effective as prophylaxis for ESI. Longer study is required to determine the long-term efficacy and the potential beneficial effect on the prevention of peritonitis.

Acute renal impairment in coronavirus-associated severe acute respiratory syndrome.

BACKGROUND: Severe acute respiratory syndrome (SARS) is a newly emerged infection from a novel coronavirus (SARS-CoV). Apart from fever and respiratory complications, acute renal impairment has been observed in some patients with SARS. Herein, we describe the clinical, pathologic, and laboratory features of the acute renal impairment complicating this new viral infection. METHODS: We conducted a retrospective analysis of the plasma creatinine concentration and other clinical parameters of the 536 SARS patients with normal plasma creatinine at first clinical presentation, admitted to two regional hospitals following a major outbreak in Hong Kong in March 2003. Kidney tissues from seven other patients with postmortem examinations were studied by light microscopy and electron microscopy. RESULTS: Among these 536 patients with SARS, 36 (6.7%) developed acute renal impairment occurring at a median duration of 20 days (range 5-48 days) after the onset of viral infection despite a normal plasma creatinine level at first clinical presentation. The acute renal impairment reflected the different prerenal and renal factors that exerted renal insult occurring in the context of multiorgan failure. Eventually, 33 SARS patients (91.7%) with acute renal impairment died. The mortality rate was significantly higher among patients with SARS and acute renal impairment compared with those with SARS and no renal impairment (91.7% vs. 8.8%) (P < 0.0001). Renal tissues revealed predominantly acute tubular necrosis with no evidence of glomerular pathology. The adjusted relative risk of mortality associated with the development of acute renal impairment was 4.057 (P < 0.001). By multivariate analysis, acute respiratory distress syndrome and age were the most significant independent risk factors predicting the development of acute renal impairment in SARS. CONCLUSION: Acute renal impairment is uncommon in SARS but carries a high mortality. The acute renal impairment is likely to be related to multi-organ failure rather than the kidney tropism of the virus. The development of acute renal impairment is an important negative prognostic indicator for survival with SARS.