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Kidney Microcirculation
Published in John H. Barker, Gary L. Anderson, Michael D. Menger, Clinically Applied Microcirculation Research, 2019
Hemolytic-uremic syndrome and sickle cell nephropathy are examples of thrombotic disorders of the renal vasculature. In the hemolytic-uremic syndrome, the renal manifestations occur abruptly as anuria, hypertension, and azotemia.9 The typical vascular lesions involve small arteries and afferent arterioles. There is intimai hyperplasia, subintimal fibrin deposition, and fibrin thrombi. In sickle cell nephropathy, the prominent renovascular lesion is RBC engorgement and thrombosis of the vasa rectae capillaries.10
Renal tumours
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
From the list of options above, choose the one that corresponds to each of the following. Each option may be used once, more than once, or not at all. Represents 6%–7% of all paediatric malignancies.Has a 3- to 5-year survival of 85%.Subtypes include papillary and clear cell types.Most common tumour in those under 3 months.Present with bilateral kidney disease in 6%.Which tumour is also known as bone metastasising tumour of childhood?Originally described as the seventh sickle-cell nephropathy.
Renal Disease in Sub-Saharan Africa
Published in Meguid El Nahas, Kidney Diseases in the Developing World and Ethnic Minorities, 2005
Ebun L. Bamgboye, Nomandla Madala, Saraladevi Naicker
This occurs even more commonly in the region with a conservative estimate of 1000 new cases per year (3). Hospital-based studies reveal that chronic renal failure (CRF) is responsible for up to 2–8% of medical admissions (3,11). The aetiology varies with the various studies but hypertensive nephro-sclerosis and chronic glomerulonephritis both predominate in all the centres (3,6,7,10,11). Other causes peculiar to the region include sickle-cell nephropathy (in West Africa) and renal tuberculosis.
An expert review of voxelotor for the treatment of hemolytic anemia in patients with sickle cell disease: ‘bridging the gap between laboratory data and patient related outcomes’
Published in Expert Review of Hematology, 2023
Baba P. D. Inusa, Khuthala Mnika, Samah Babiker
Renal pathology, closely tied to anemia, shows some early evidence of disease amelioration. Sickle cell nephropathy includes a range of tubular and glomerular abnormalities. While the underlying mechanisms of renal injury in SCD are largely related to vaso-occlusion, hemolysis, via toxic effects of cell-free plasma hemoglobin on the renal vasculature, may also play a role as has been seen in animal models. Albuminuria, defined as albumin/creatinine ratio of 30 mg/g, is both an early manifestation of kidney dysfunction in SCD that progresses with age. In five patients with SCA and chronic kidney disease (CKD) stages 1–3, Han et al. showed a decrease in urine albumin concentration from baseline and compared to untreated controls [18]. While it is difficult to draw conclusions from this small study, this data is reassuring given the morbidity and mortality associated with CKD in patients with SCD and the lack of alternative targeted options. In addition, no adverse events, namely renal pathology progression, were noted.
Assessment of cystatin C in pediatric sickle cell disease and β-thalassemia as a marker of subclinical cardiovascular dysfunction: a case-control study
Published in Pediatric Hematology and Oncology, 2021
Diana Hanna, Mohamed Beshir, Naglaa Khalifa, Eman Baz, Ahmed Elhewala
Hemoglobinopathies, such as thalassemia and sickle cell disease (SCD), are highly prevalent genetic disorders with a significant health burden worldwide.1 Sickle hemoglobin (HbS) is a structural variant of normal adult hemoglobin (HbA).2 SCD is characterized by erythrocyte sickling, chronic hemolytic anemia, episodic acute vaso-occlusion and cumulative organ damage.3 Chronic anemia in SCD results in cardiac dilatation and a compensatory increase in left ventricular mass, this is often accompanied by left ventricular diastolic dysfunction.4 With improved childhood survival, SCD has evolved into a chronic degenerative disease with underlying damage to multiple organs including the heart.5 Cardiopulmonary complications, including cardiomyopathy, diastolic dysfunction, pulmonary hypertension (PHT), and sudden cardiac death are the most common causes of morbidity and mortality in SCD.5 Sickle cell nephropathy is a major complication of SCD causing tubular and medullary dysfunction as well as renal failure due to nephron loss.6 Preclinical markers of glomerular damage as micro-albuminuria can be measured as early predictors of progressive renal nephropathy.7