Screening for Fabry disease among 619 hemodialysis patients in Saudi Arabia.

OBJECTIVES: To determine the prevalence of Fabry disease (FD) among Saudi patients on hemodialysis. METHODS: This prospective study was conducted in 3 major hospitals in the Kingdom of Saudi Arabia (KSA). All adult patients (greater than 18 years old) attending the dialysis unit who have end-stage renal disease (ESRD) and on hemodialysis were included. Known patients with FD and those who refused to participate in the study were excluded. All eligible patients were screened for FD using dry blood spot (DBS) for alpha-galactosidase A (α-Gal A). A positive DBS (enzyme activity less than 40%) was followed by another con rmatory enzyme assay. When the second DBS sample was also positive (enzyme activity less than 40%), a Sanger sequencing of the GLA gene was performed. RESULTS: A total of 619 patients with ESRD and on hemodialysis were screened for FD using DBS for α-Gal A enzyme level. Enzymatic activity was below 40% in 11 samples. On retesting, 3 females had less than 20% enzymatic activity suggesting FD. Sanger sequencing of these 3 females showed the variant c.1055C greater than G (p.Ala352Gly) confirming the diagnosis of FD. Family screening of one of these 3 patients revealed one asymptomatic female carrying the same variant. CONCLUSION: The prevalence of FD in this cohort was 4.8 per 1000 patients. Screening of Fabry patients with ESRD seems to be a cost-effective strategy. Furthermore, relatives of the patients identified by screening enhances this screening strategy.

Awareness and Impact of Non-pharmaceutical Interventions During Coronavirus Disease 2019 Pandemic in Renal Transplant Recipients.

The concerns generated by coronavirus disease 2019 (COVID-19) pandemic are having profound impact on solid organ transplantation (SOT). Non-pharmaceutical interventions (NPI) are currently the only measures available to contain COVID-19 in the general population and in more vulnerable recipients of any organ transplant. In this cross-sectional case control study from a patient survey undertaken in 2 transplant centers (TxC) in the Kingdom of Saudi Arabia and Italy, we aimed to appraise awareness of the NPI implemented by respective these governments. We have also evaluated the impact of COVID-19 on our kidney transplant (KT) recipients and a control group of kidney living donors (KLD). In our series, there were zero cases of COVID-19 among 111 KT recipients and 70 KLD of the control group. Demography, transplant type, immunosuppression regimes, and, importantly, the different COVID-19 prevalence in the 2 regions of the TxC did not appear to influence incidence of COVID-19 in our KT recipients. The absence of COVID-19 cases in our series was unexpected. Our findings suggest that awareness of NPI is associated with a successful containment of COVID-19 in vulnerable, immunosuppressed KT recipients.

Steroid-Sparing and Steroid-Based Immunosuppression in Kidney Transplant: Is There a Difference in Outcomes and Recipient Comorbidities?

OBJECTIVES: Corticosteroids are fundamental to immunosuppression in kidney transplant but have significant side effects, generating interest in steroid-sparing immunosuppression regimens. We studied corticosteroid withdrawal on graft outcomes and comorbidities to study individualized approaches for immunosuppression. This is the first study of its kind in our ethnically distinct population. MATERIALS AND METHODS: Of 103 consecutive Saudi kidney transplant recipients seen in our unit during 2014-2018, 102 passed screening; 32 received no oral steroids after immunosuppression induction (steroid-spared group) and 70 received standard steroid-based immunosuppression. Both groups had similar immunosuppression induction: the standard steroid based immunosuppression included oral prednisolone (30 mg tapered to 5 mg over 6 wk), tacrolimus, and mycophenolate mofetil; however, the steroid-spared group did not receive corticosteroids after methylprednisolone induction. The Mann-Whitney U test compared numerical data, and the Fisher exact test compared categorical data. Relative risks for adverse events, graft dysfunction, and high serum creatinine were calculated. P < .05 was considered significant. RESULTS: Compared with the steroid-based group, patients in the steroid-spared group were older, had higher mean body mass index, and more favorable human leukocyte antigen matching and panel reactive antibody profiles. Mean serum creatinine and proportion of recipients with above normal serum creatinine were greater in the steroid-based group; this group also had slightly higher incidence of acute rejection. No graft failures or recipient deaths occurred in either group. The steroid-based group had significantly greater weight gain than the steroid-spared group (67% vs 34%; P = .002). The steroid-spared group exhibited better control of blood pressure and serum lipids; however, this was not statistically significant. CONCLUSIONS: Early steroid withdrawal in selected transplant recipients is a viable option for immunosuppression, with no compromised graft function or survival shown in our cohort. Given the significant impact of weight gain, blood pressure, and serum lipids on recipient morbidity and mortality, a larger study is warranted.

Implantation Warm Ischemia Time in Kidney Transplant Recipients: Defining Its Limits and Impact on Early Graft Function.

BACKGROUND Prolonged cold ischemia is an established risk factor for poor early graft function (EGF). However, warm ischemia incurring during graft implantation has received little attention regarding its possible detrimental effect on EGF. The aim of our study was to examine the impact of recipient warm ischemia time on EGF. MATERIAL AND METHODS The data of 102 consecutive kidney transplants were analyzed to determine the association between duration of graft implantation time (IT) and EGF. Recipient IT groups were (GI) up to 45 min, (GII) 45-60 min, and (GIII) >60 min. EGF was categorized as immediate (IGF), slow (SGF), or delayed graft function (DGF). In recipients with IGF, graft function was further assessed by time needed for reduction in serum creatinine by 50% (SC50) of pre-transplant value, and serum creatinine on day 7 (SCD7). RESULTS Of a total of 102 recipients, 55 (55%) were in GI, 33 (32%) were in GII, and 14 (13%) were in GIII. Factors prolonging IT were recipient body mass index (BMI) (p=0.02) and multiple arteries in donor kidneys (p<0.01). No recipients in GI had DGF or SGF, while 2 in GII had DGF, and 5 patients in GIII had poor EGF. SC50 was significantly longer in GIII and GII versus GI (40.8±42.4 and 32.8±20.4 vs. 22.2±17.2 [p=.02, p≤.01]), respectively. Mean SCD7 was also significantly higher in GIII and GII versus GI. The mean last serum creatinine was comparable among all groups. CONCLUSIONS IT of more than 45 min was a risk factor for poor EGF, but achieved statistical significance only when it exceeded 60 min. Longer IT also significantly slowed the fall in SC50, and led to a higher SCD7. However, poor EGF and suboptimal early SC trends had little long-term effect on serum creatinine.

Implantation of Right Kidneys: Is the Risk of Technical Graft Loss Real?

BACKGROUND: The left kidney (LK) is preferred by transplant surgeons, because its vein is always of good length and has a thick wall that enables safe suturing. On the other hand, the right renal vein is generally shorter and thinner walled, and well known for its technical difficulty during venous anastomosis, and can result in graft loss. We examined our living (LD) and deceased donor (DD) recipient data and compared the incidence of technical graft loss and early graft function in right and left kidneys. METHODS: A cohort of 58 adult and pediatric recipients received an LD or DD kidney between January 2015 and December 2016. The donor and recipient data were retrieved and retrospectively analyzed. Technical graft loss was defined as graft thrombosis within the 7 days after transplant. RESULTS: Right kidneys (RKs) were not a risk factor for technical graft loss, and no graft was lost for technical reasons in either LD or DD transplants. Early graft function in LK and RKs was also comparable in the LD cohort, and there were no LKs in the DD cohort. CONCLUSION: Based on our data, the use of RKs was not a risk factor for technical graft loss and early graft function was comparable to LKs.