Primary Renal Synovial Sarcoma Presenting as Haemorrhagic Shock: A Rare Presentation

Primary synovial sarcoma (PSS) of the kidney is considered the rarest type of all renal sarcomas with specific chromosomal translocation t (X; 18) (p11.2; q11.2). We report the case of a 65-year-old man with no medical conditions who presented to the emergency department with sudden severe right flank pain associated with haemodynamic instability and haemorrhagic shock. Computed tomography (CT) of the abdomen and pelvis revealed a right renal mass. A right open radical nephrectomy was performed. Histopathology revealed a monophasic synovial sarcoma. The patient received six cycles of docetaxel and gemcitabine as adjuvant chemotherapy. No sign of recurrence was seen on a follow-up CT urogram. This rare tumour often presents atypically, and clear guidelines regarding appropriate treatment are lacking. Our case showed that treatment with docetaxel/gemcitabine after an open radical nephrectomy is promising

Epidemiology profile of renal cell carcinoma: A 10-year patients' experience at King Abdulaziz Medical City, National Guard Health Affairs, Saudi Arabia

Purpose: The purpose of this study is to describe the epidemiological profile, histopathological features, and outcomes of patients diagnosed with renal cell carcinoma (RCC) in a tertiary referral center over 10 years. Methodology: This is a retrospective cohort of 219 Saudi patients diagnosed with RCC between June 2003 and May 2013. The variables collected included the sociodemographic details and clinical presentation. The histopathological features investigated include the tumors histological subtype, pathologic staging tumor, node, and metastasis descriptors, and lymph-vascular invasion. Patients were followed until May 2013. Bivariable analysis was calculated using Chi-square test, with level of significance set at P < 0.05. Kaplan–Meier estimate was used to calculate the survival rate. Results: The mean age of patients was 57.18 (±14.68 standard deviation). The trend of patients diagnosed with RCC over the past 10 years was higher among males than females (60.27% vs. 39.73%). Noticeably, more than half (57.58%) were diagnosed incidentally. The most common histological subtype was clear cell (conventional) RCC (70.44%). Patients were usually diagnosed at the pT1 stage (48.1%). The histopathological features associated with worse patient outcome were the stage of the primary tumor (P = 0.01) and lymph-vascular invasion (P = 0.003). The overall mean survival rate was 2.03 years. Conclusion: In the past 10 years, there are more patients diagnosed incidentally with RCC, which is in line with the global trend. Patients were more likely to be male and middle aged. We recommend further population-based studies in this area to establish a national epidemiological data for this common type of cancer

Factors impeding the supply of over-the-counter medications according to evidence-based practice: A mixed-methods study.

ObjectiveDespite the positive attitudes pharmacists have toward evidence-based practices (EBPs), its application in community pharmacies in Saudi Arabia is lacking. Therefore, this study aimed to explore and assess EBPs by community pharmacists in Saudi Arabia when they dispense over-the-counter (OTC) medications for three minor ailments: diarrhea, cough, and the common cold.Research design and methodsWe used a mixed-methods approach consisting of two study parts. The first was a quantitative investigation that used mystery shoppers. Four researchers, posing as mystery shoppers, visited 214 randomly selected pharmacies in the Riyadh region of Saudi Arabia. They used 14 questions from a standardized checklist to examine EBPs by community pharmacists. The qualitative part of the study entailed three focus-group discussions with 13 pharmacists from different community practice settings and explored factors that affected the application of EBPs when supplying OTC medications from the pharmacists' point of view.ResultsThe analysis indicated that 40% of pharmacists dispensed OTC medications according to EBPs. Logistic regression analysis showed that one question, "Describe your symptoms", predicted the correct supply of OTC medications (p = 0.021). The qualitative section of the study identified nine factors that affected EBP. Some of these factors facilitated EBP, such as established patient-pharmacist relationships, some acted as barriers such as conflicts between available evidence, while other factors could either facilitate or hinder EBPs, such as the health literacy of the patient.ConclusionGiven that dispensing OTC medication is a core function of pharmacists, this study uncovered low adherence to EBPs by community pharmacists in Saudi Arabia when dispensing OTC medication for three minor ailments: diarrhea, cough, and the common cold. Furthermore, this study identified a number of explanatory factors for this low adherence. Targeting these factors could help change the behavior of pharmacists and decrease undesirable outcomes

Atypical antiglomerular basement membranes disease with nephrotic-range proteinuria, mesangial proliferation, and membranoproliferative glomerulonephritis pattern of injury

Antiglomerular basement membrane (anti-GBM) disease is an uncommon autoimmune disease characterized by the presence of IgG autoantibodies targeting the alpha-3 chain of type IV collagen. Some of the atypical forms of the disease have been described. Herein, we describe a case of atypical anti-GBM in a 27-year-old Saudi male who presented with lower limb edema, gross hematuria, elevated serum creatinine concentration, and nephrotic-range proteinuria. All serology tests were negative, except for anti-GBM which was weakly positive. Renal biopsy showed proliferative glomerulonephritis (GN) with nodular transformation of the glomerular tufts, mesangial hypercellularity (mesangial cell proliferation), segmental endocapillary hypercellularity and three incomplete cellular crescents, and recapitulating membranoproliferative GN pattern of glomerular injury. Direct immunofluorescence microscopy demonstrated diffuse, intense linear positivity for IgG and Kappa and Lambda light chains, and compatible with anti-GBM disease. The patient was treated with cyclophosphamide and corticosteroids in addition to therapeutic plasma exchange which resulted in mild improvement in renal function over a period of six weeks. We emphasize the importance of recognition of atypical pathological and serological patterns of anti-GBM disease, which is crucial for proper and early diagnosis and possibly improved clinical outcome and we highlight the importance of clinicopathological correlation in cases with atypical clinical and pathological presentations

Alport’s syndrome with focal segmental glomerulosclerosis lesion – Pattern to recognize

The association between Alport’s syndrome (AS) and focal segmental glomerulosclerosis (FSGS) in the same patient is complex and rarely reported. We report a case of a 42-year-old male presenting with proteinuria, microscopic hematuria, elevated serum creatinine and hypertension with unremarkable physical examination apart from obesity. The renal biopsy showed well-established FSGS pattern of injury with mild interstitial fibrosis and tubular atrophy, while the electron microscopic examination demonstrated glomerular basement membranes (GBM) changes compatible with AS. AS can be complicated by segmental glomerular scarring, which can mimic primary FSGS, while familial FSGS can result from mutations in collagen IV network of the GBM. This overlap can complicate histopathological interpretation of renal biopsy, which should be accompanied by mutational analysis for accurate diagnosis and proper therapeutic intervention

Herb-induced acute bone marrow intoxication and interstitial nephritis superimposing glomerular C1q deposition in a patient with paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria is a rare disease of the red blood cell membrane that renders it lyzable by the complement system, leading to chronic intravascular hemolysis. Renal hemosiderosis is a well-known complication of intravascular hemolytic anemia and can lead to acute kidney injury and renal failure. The use of herbal medicine is common worldwide. The nephrotoxicity of herbal remedies can take several forms, which include acute kidney injury and acute and chronic interstitial nephritis. In addition, the use of herbal remedies can result in bone marrow toxicity and suppression. C1q nephropathy is an uncommon form of glomerular disease characterized by dominant or co-dominant glomerular immunofluorescence positivity for C1q in the absence of clinical and serological evidence of systemic lupus erythematosus, and has various clinical presentations and outcome. Here, we report a patient of undiagnosed paroxysmal nocturnal hemoglobinuria who consumed herbal medicine of unknown constituents and clinically presented with anemia and acute kidney injury. The pathological findings of bone marrow and renal biopsies that include bone marrow intoxication, severe renal hemosiderosis and acute interstitial nephritis and kidney injury, as well as co-dominant glomerular deposition of C1q, are discussed. In addition, we discuss and hypothesize the possible pathogenesis of glomerular C1q deposition in the setting of paroxysmal nocturnal hemoglobulinuria