Sickle cell disease (SCD), globally regarded as one of the most prevalent hereditary diseases of the hematologic system, stems from mutations of genes (inherited through an autosomal recessive pattern) responsible for the formation of hemoglobin, specifically the β-globin chain [1] [2]. Depending on the phenotype, the medical entity may have a very poor prognosis in homozygous forms, whereas compound heterozygotes, as well as the heterozygotes, carry a slightly better life expectancy with less severe clinical manifestations [1]. The pathogenesis of SCD is centered around the defective hemoglobin molecules, which bind together in the circulation causing obstruction of the blood flow, sludging, and increased blood viscosity [3] [4]. As a result, ischemia and hypoxia of many tissues may arise. Renal failure, occurring on the grounds of microvascular obstruction, medullary fibrosis, focal segmental glomerulosclerosis (FSGS), and papillary necrosis, is known as sickle cell nephropathy (SCN), affecting up to 18% of all SCD cases [3] [4] [5]. Signs of SCN can start as early as infancy, when the capacity for urine concentration is diminished, resulting in polyuria and dehydration [3] [5] [6] [7]. In addition, microalbuminuria and proteinuria, as well as hematuria, anemia, and hypertension are main symptoms of SCN [3] [5] [6] [7]. In virtually all patients, progressive deterioration of glomerular filtration rate (GFR) increases the risk of acute kidney injury (AKI), chronic kidney disease (CKD, seen in up to 39% of SCD cases), and end-stage renal disease (ESRD) [1] [3] [5] [6] [8].

• Polydipsia

  In this case the diagnosis is irreversible nephrogenic diabetes insipidus, whose most common clinical manifestations are nocturia, polyuria, and polydipsia. 2. Distal tubular acidosis. [revistanefrologia.com]

• Neglect

  Retarding progression of chronic renal disease; the neglected issue of residual proteinuria. Kidney Int 2003;63:2254-61. 9. Brenner BM, Cooper ME, de Zeeuw D, Keane WF, Mitch WE, Parving HH, et al. [sjmms.net]

• Nocturia

  […] negative renal failure increased mortality risk for those with renal failure, regardless of treatment prognosis is 1 year for renal medullary carcinoma commonly very aggressive and metastasizes early Presentation Symptoms vaso-occlusion nausea vomiting nocturia [medbullets.com]

  HyposthenuriaHyposthenuria 1st clinical evidence of defective medullary1st clinical evidence of defective medullary tonicitytonicity Output 2000ml/day, nocturia andOutput 2000ml/day, nocturia and enuresisenuresis Max concentrating ability falls toMax [slideshare.net]

  Improving nocturia has been reported to be an additional beneficial effect of ACEIs, presumably as a result of reduction in GFR. [emedicine.medscape.com]

  In this case the diagnosis is irreversible nephrogenic diabetes insipidus, whose most common clinical manifestations are nocturia, polyuria, and polydipsia. 2. Distal tubular acidosis. [revistanefrologia.com]

The diagnosis of sickle cell nephropathy can be made through a comprehensive clinical approach and subsequent laboratory testing. After collection of anamnestic data, during which findings such as hematuria and polyuria (and weakness due to dehydration and anemia) are noticed, urinalysis and additional imaging studies need to be performed. Urine dipstick testing is a useful initial tool to detect proteinuria and more detailed exams that assess the extent of protein loss are mandatory [3]. Further urinalysis reveals hematuria, in which case ultrasonography of the kidneys and the urinary bladder, cystoscopy, or urography, might be necessary to exclude other diagnoses (eg. the presence of stones or other lesions ) [2] [6]. Hyperechogenic renal pyramids and clubbing of the renal calyces are highly suggestive of SCN on ultrasonography and urography, respectively [5]. Serum creatinine and blood urea nitrogen (BUN) are important components of the workup, mainly in order to calculate the GFR and establish the severity of kidney disease. Erythropoietin (EPO) levels are markedly reduced in patients with sickle cell nephropathy, particularly in severe stages of renal failure [6]. To identify SCD as the underlying etiology of nephropathy, the aberrant hemoglobin (HbS) must be recognized through procedures such as isoelectric focusing (IEF), genetic studies, or high-performance liquid chromatography [2].

• Penicillium

  The pathogenesis is poorly understood, hence the retention of the title nephropathy. mycotoxic nephropathy caused by mycotoxins, such as ochratoxin and citrinin, produced by Penicillium and Aspergillus spp. reflux nephropathy pyelonephritis in which the [medical-dictionary.thefreedictionary.com]

1. Nath KA, Hebbel RP. Sickle cell disease: renal manifestations and mechanisms. Nat Rev Nephrol. 2015;11(3):161-171.
2. Bender MA, Douthitt Seibel G. Sickle Cell Disease. 2003 Sep 15 [Updated 2014 Oct 23]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2017.
3. Saborio P, Scheinman JI. Sickle cell nephropathy. J Am Soc Nephrol. 1999;10(1):187-192.
4. Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. Radiographics. 2001;21(4):971-994.
5. Pham PT, Pham PC, Wilkinson AH, Lew SQ. Renal abnormalities in sickle cell disease. Kidney Int. 2000;57(1):1-8.
6. Ataga KI, Orringer EP. Renal abnormalities in sickle cell disease. Am J Hematol. 2000;63(4):205-211.
7. Alhwiesh A. An update on sickle cell nephropathy. Saudi J Kidney Dis Transpl. 2014;25(2):249-265.
8. Ephraim RKD, Osakunor DNM, Cudjoe O, et al. Chronic kidney disease is common in sickle cell disease: a cross-sectional study in the Tema Metropolis, Ghana. BMC Nephrol. 2015;16:75.