Epidemiology, Management, and Outcome of Atypical Hemolytic Uremic Syndrome in an Omani Cohort.

Objectives: Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening disease of chronic uncontrolled complement pathway activation that leads to thrombotic-microangiopathy, along with severe organ damage, including end-stage kidney disease. This study aimed to evaluate the epidemiology, management, and outcome of aHUS in an Omani population. Methods: This retrospective descriptive cohort study assessed all cases of aHUS diagnosed and followed up at two tertiary care centers in Oman from January 2008 to December 2019, based on clinical features, complement pathway assays, histopathological, and genetic testing. Results: The study accrued 19 patients who fulfilled the inclusion criteria, of whom 11 (57.9%) were male. The participants' median age was 25.0 years (range = 0.1-69.0). Most (15; 78.9%) patients presented in the acute phase of the disease. The triad of hemolytic anemia, acute kidney injury, and thrombocytopenia was present in all patients. A trigger factor (e.g., infection) was identified in 68.4% of cases. Of the 14 (73.7%) patients who underwent kidney biopsy, 10 (71.4%) were found to have aHUS in native kidneys and three in grafted kidneys. Of the 11 (57.9%) patients who underwent genetic analysis, five (45.5%) were found to have a known pathogenic variant in their aHUS susceptibility genes. Plasma exchange followed by eculizumab was the treatment method in 11 (57.9%) cases. Complete renal recovery was achieved in seven (36.8%) patients, while four (21.1%) passed away during the study period. Conclusions: The wide spectrum and multiple expressions of aHUS make it a challenge to diagnose and consequently may delay the commencement of the targeted treatment. Eculizumab is considered the first-line therapy and should be commenced as early as possible.

"Virtual Interdisciplinary COVID-19 Team": A Hospital Pandemic Preparedness Approach.

The COVID-19 pandemic continues to move at record speed. Health systems and hospitals worldwide face unprecedented challenges to effectively prepare and respond to this extraordinary health crisis and anticipated surge. Hospitals should confront these unparalleled challenges with a comprehensive, multidisciplinary, coordinated, and organized strategy. We report our experience with the systematic application of the "4S" principle to guide our institutional preparedness plan for COVID-19. We used an innovative "virtual interdisciplinary COVID-19 team" approach to consolidate our hospital readiness.

Successful Salvage Treatment of Resistant Acute Antibody-Mediated Kidney Transplant Rejection with Eculizumab.

Antibody-mediated rejection (ABMR) jeopardises short- and long-term transplant survival and remains a challenge in the field of organ transplantation. We report the first use of the anticomplement agent eculizumab in Oman in the treatment of a 61-year-old female patient with ABMR following a living unrelated kidney transplant. The patient was admitted to the Sultan Qaboos University Hospital in Muscat, Oman, in 2013 on the eighth day post-transplantation with serum creatinine (Cr) levels of 400 µmol/L which continued to rise, necessitating haemodialysis. A biopsy indicated ABMR with acute cellular rejection. No improvement was observed following standard ABMR treatment and she continued to require dialysis. Five doses of eculizumab were administered over six weeks with a subsequent dramatic improvement in renal function. The patient became dialysis-free with serum Cr levels of 119 µmol/L within four months. This case report indicates that eculizumab is a promising agent in the treatment of ABMR.

Safety and Complications of Double-Lumen Tunnelled Cuffed Central Venous Dialysis Catheters: Clinical and radiological perspective from a tertiary centre in Oman.

OBJECTIVES: This study aimed to assess the technical success, safety and immediate and delayed complications of double-lumen tunnelled cuffed central venous catheters (TVCs) at the Sultan Qaboos University Hospital (SQUH), Muscat, Oman. METHODS: This retrospective study took place between January 2012 and October 2013. The clinical records and radiological data of all patients who underwent ultrasound- and fluoroscopy-guided TVC placement at SQUH during the study period were reviewed. Demographic data and information regarding catheter placement, technical success and peri- and post-procedure complications (such as catheter-related infections or thrombosis) were collected. RESULTS: A total of 204 TVCs were placed in 161 patients. Of these, 68 were female (42.2%) and 93 were male (57.8%). The mean age of the patients was 54.4 ± 17.3 years. The most common reason for catheter placement was the initiation of dialysis (63.4%). A total of 203 procedures were technically successful (99.5%). The right internal jugular vein was the most common site of catheter placement (74.9%). Mild haemorrhage which resolved spontaneously occurred in 11 cases (5.4%). No other complications were observed. Subsequent follow-up data was available for 132 catheters (65.0%); of these, thrombosis-related catheter malfunction was observed in 22 cases (16.7%) and catheter-related infection in 29 cases (22.0%). CONCLUSION: Radiological-guided placement of tunnelled haemodialysis catheters can be performed safely with excellent technical success. The success rate of catheter insertion at SQUH was favourable in comparison with other studies reported in the literature.

Chronic renal failure presenting for the first time as pulmonary mucormycosis with a fatal outcome.

Pulmonary mucormycosis is an uncommon, but important, opportunistic fungal pneumonia which is often diagnosed late. Renal failure as the predominant presenting feature is not common in mucormycosis. Moreover, sudden, massive hemoptysis is not a usual complication. In this report we describe fatal pulmonary mucormycosis in a young patient with a previously undiagnosed chronic renal failure.

Acute Allograft Renal Vein Thrombosis: A Case Report.

Renal graft thrombosis is a serious and devastating complication of renal transplant that ultimately results in graft loss. It is associated with acute and hyper-acute rejections; however, the underlying cause in large proportion of patients remains unknown. We report a case of a young male who underwent live related kidney transplant but lost the graft on the operating table due to renal vein thrombosis (RVT).

The spectrum of glomerular diseases on renal biopsy: data from a single tertiary center in oman.

OBJECTIVE: To study the pattern of glomerular disease (GD) from the result of renal biopsies at our center. METHODS: We conducted a retrospective review of 190 adult native renal biopsy reports from the pathology registry of renal biopsy performed at our hospital between 1992 and 2010. RESULTS: Lupus nephritis was the most common pathology 48/133 (36.1%) with a female preponderance. The most common primary glomerular disease was focal segmental glomerulosclerosis (FSGS) 26/133(19.5%), followed by membranous glemerulopathy (MGN) 13/133 (9.8%), and mesangial proliferative glomerulonephritis 6/133 (4.5%). IgA nephropathy and acute proliferative glomerulonephritis each accounted for 4/133 (3.0%). Membranoproliferative glomerulonephritis accounted for 3/133 (2.3%). Focal proliferative and cresentic glomerulonephritis each accounted for 2/133 (1.5%). Vasculitis was not common and there was no report of anti-GBM disease. CONCLUSION: Among the secondary glomerular diseases, lupus nephritis was the commonest condition with a female preponderance. Among the primary glomerular diseases, FSGS was the commonest. These results are consistent with global trend. IgA nephropathy is not common as the case in the Caucasian population. Vasculitis was not common and there was no report of anti-GBM disease.

Prevalence and risk factors of diabetic nephropathy in omani type 2 diabetics in Al-dakhiliyah region.

OBJECTIVE: To assess the prevalence and risk factors of diabetic nephropathy among Omani type 2 diabetics in Al-Dakhiliyah region of the Sultanate of Oman. METHODS: A cross-sectional and a case control study designs were used to assess the prevalence and risk factors respectively. For the prevalence study a sample of 699 diabetic subjects were selected randomly from two polyclinics in Al-Dakhiliyah region; Sumail and Nizwa polyclinics. For the case control study, a sample consisting of 215 cases and 358 controls were randomly selected from those who were included in the cross-sectional study. A well designed questionnaire has been used to collect data regarding the disease and risk factors. Data was analyzed using SPSS19 statistical program. RESULTS: Total prevalence of diabetic nephropathy was calculated as 42.5% (95% C.I: 38.83% - 46.15%). The difference in the prevalence in the two polyclinic catchment area was not significant. The prevalence was significantly higher among males (51.6%) compared to females (36.5%). Crude analysis of the risk factors showed significant association between diabetic nephropathy and the following factors; male gender, decreased literacy, long duration of diabetes mellitus, hypertension, retinopathy, neuropathy, family history of diabetic nephropathy, poor glycemic control (high HbA1c), and hypertriglyceridemia. Multivariate analysis showed the following factors to be independent risk factors; male gender, decreased literacy, long duration of diabetes, family history of diabetic nephropathy and poor glycaemic control (high HbA1c). CONCLUSION: The prevalence of diabetic nephropathy in this study was 42.5% and the significant risk factors associated with it included male gender, decreased literacy, long duration of diabetes, family history of diabetic nephropathy and poor glycemic control (high HbA1c).

Estimated Glomerular Filtration Rate (eGFR): A Serum Creatinine-Based Test for the Detection of Chronic Kidney Disease and its Impact on Clinical Practice.

Chronic kidney disease (CKD) is an important epidemic and public health problem that is associated with a significant risk for vascular disease and early cardiovascular mortality as well as progression of kidney disease. Currently it is classified into five stages based on the glomerular filtration rate (GFR) as recommended by many professional guidelines. Radiolabelled methods for measuring GFR are accurate but not practical and can be used only on a very limited scale while the traditional methods require timed urine collection with its drawback of inaccuracy, cumbersomeness and inconvenience for the patients. However, the development of formula- based calculation of estimated GFR (eGFR) has offered a very practical and easy approach for converting serum creatinine value into GFR result taking into consideration patient's age, sex, ethnicity and weight (depending on equation type). The commonly used equations include Cockraft and Gault (1976), Modification of Diet in Renal Disease (MDRD) (1999) and Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) (2009). It is the implementation of these equations particularly the MDRD that has raised the medical awareness in the diagnosis and management of CKD and its adoption by many guidelines in North America and Europe. The impact and pitfalls of each of these equations in the screening, diagnosis and management of patients with CKD are presented and discussed in this review.

Infective Endocarditis of the Aortic Valve caused by Pseudomonas aeruginosa and Treated Medically in a Patient on Haemodialysis.

Infective endocarditis (IE) in patients on dialysis is a serious infection with a high mortality rate. It is usually caused by Gram positive bacteria with Gram negative organisms being relatively rare as a cause. Recommended treatment usually involves surgical valve replacement and the extended use of antibiotics. Successful treatment with antibiotics alone is rare. We report a case of IE caused by Pseudomonas aeruginosa in a patient on dialysis treated solely with antibiotics.

Transplant renal artery false aneurysm: case report and literature review.

This is a case report of a 59 years old male who had a commercial non-related living renal transplantation for his end stage renal insufficiency secondary to adult polycystic kidney disease. He suffered an immediate and early post-operative bleeding, which was managed conservatively. He was presented at Sultan Qaboos University Hospital four months after his transplant with abdominal pain, nausea, loss of appetite and a rise in serum creatinine levels. Ultrasonography and angiography have shown a 4 cm false aneurysm of the transplant renal artery at the anastomotic site with the external iliac artery. Surgical exploration with resection of the false aneurysm and reanastomosis of the donor renal artery to the external iliac artery was carried out successfully with preservation of the renal allograft. This is a rare case of an extra-renal false aneurysm at the anastomotic site of the transplant renal artery to the external iliac artery four months after renal allotransplantation. Literature review on the management and outcome of false aneurysms after renal transplant was carried out.

Neurofibromatosis with bilateral renal artery stenosis and end stage renal disease: case report.

Neurofibromatosis type 1 (NF-1) is an autosomal dominant, hereditary, neurocutaneous syndrome that may, primarily or secondarily, affect different organs or systems of the body including the cardiovascular system. The most common vascular abnormality in patients with NF-1 is renal artery stenosis. Here we report the case of a middle-aged gentleman who presented at Sultan Qaboos University Hospital, Oman, with end stage renal disease and severe hypertension and was diagnosed to have NF-1 with bilateral renal artery stenosis. He was started on renal replacement therapy.

Decreased survival in liver transplant patients requiring chronic dialysis: a Canadian experience.

BACKGROUND: Chronic kidney disease is associated with increased mortality among nonrenal organ transplant recipients. End-stage renal disease (ESRD) is a serious complication after orthotopic liver transplantation (OLT). It is unclear if the outcomes of these individuals are different from nontransplant patients requiring dialysis or a kidney transplant. METHODS: We report the incidence of ESRD in OLT recipients and compare their outcomes to matched dialysis controls. We analyzed 4186 patients who received an OLT in Canada between January 1981 and December 2002 and 228 matched, nontransplant, chronic dialysis controls. RESULTS: The incidence of ESRD after OLT was 2.9% (n=120). The unadjusted mortality rate for those who required chronic dialysis was 49.2% compared with 26.8% in those who did not develop kidney failure (P<0.0001). The survival of OLT recipients on dialysis was lower than the matched chronic dialysis cohort (log-rank test, P=0.01). A kidney transplant was performed in 24% of the OLT recipients and 21% of the matched dialysis cohort, and their overall survival was similar. The OLT patients who remained on dialysis had a significantly lower survival when compared with matched dialysis patients who did not receive a kidney transplant (log-rank test, P=0.0002). CONCLUSIONS: Mortality was greater for OLT recipients on dialysis than would be expected from a matched, nontransplant, dialysis cohort. Kidney transplantation may abrogate some of this increased mortality risk.