**1. Introduction**

28 Novel Insights on Chronic Kidney Disease, Acute Kidney Injury and Polycystic Kidney Disease

Steinhardt, G. F., Salinas-Madrigal, L., deMello, D., Farber, R., Phillips, B., & Vogler, G.

Strand, W. R. (2004). Initial management of complex pediatric disorders: prunebelly syndrome, posterior urethral valves. *Urol Clin North Am, 31*(3), 399-415, vii. Straub, E., & Spranger, J. (1981). Etiology and pathogenesis of the prune belly syndrome.

Thiruchelvam, N., Wu, C., David, A., Woolf, A. S., Cuckow, P. M., & Fry, C. H. (2003).

phenotypes of angiotensinogen nullizygotes. *J Clin Invest, 101*(4), 755-760. University of Birmingham. (2011). PLUTO (Percutaneous shunting in Lower Urinary Tract

van Gool, J. D., Dik, P., & de Jong, T. P. (2001). Bladder-sphincter dysfunction in

Vidal, I., Heloury, Y., Ravasse, P., Lenormand, L., & Leclair, M. D. (2009). Severe bladder dysfunction revealed prenatally or during infancy. *J Pediatr Urol, 5*(1), 3-7. Warshaw, B. L., Hymes, L. C., Trulock, T. S., & Woodard, J. R. (1985). Prognostic features in

Weide, L. G., & Lacy, P. E. (1991). Hereditary hydronephrosis in C57BL/KsJ mice. *Lab Anim* 

Wiesel, A., Queisser-Luft, A., Clementi, M., Bianca, S., & Stoll, C. (2005). Prenatal detection

Woodhouse, C. R., Ransley, P. G., & Innes-Williams, D. (1982). Prune belly syndrome--

Woodward, M., & Frank, D. (2002). Postnatal management of antenatal hydronephrosis. *BJU* 

Woolf, A. S., & Thiruchelvam, N. (2001). Congenital obstructive uropathy: its origin and

Yiee, J., & Wilcox, D. (2008). Management of fetal hydronephrosis. *Pediatr Nephrol, 23*(3), 347-

Ylinen, E., Ala-Houhala, M., & Wikstrom, S. (2004). Prognostic factors of posterior urethral valves and the role of antenatal detection. *Pediatr Nephrol, 19*(8), 874-879. Yokoyama, H., Wada, T., Kobayashi, K., Kuno, K., Kurihara, H., Shindo, T., & Matsushima,

Yoon, J. Y., Kim, J. C., Hwang, T. K., Yoon, M. S., & Park, Y. H. (1998). Collagen studies for pediatric ureteropelvic junction obstruction. *Urology, 52*(3), 494-497.

assessment. *J Urol, 152*(6 Pt 1), 2133-2138.

*Kidney Int, 20*(6), 695-699.

http://www.pluto.bham.ac.uk

240-243.

*Sci, 41*(5), 415-418.

*Int, 89*(2), 149-156.

353.

myelomeningocele. *Eur J Pediatr, 160*(7), 414-420.

report of 47 cases. *Arch Dis Child, 57*(11), 856-859.

nephropathy. *Nephrol Dial Transplant, 17 Suppl 9*, 39-41.

(1994). Experimental ureteral obstruction in the fetal opossum: histologic

Neurotransmission and viscoelasticity in the ovine fetal bladder after in utero bladder outflow obstruction. *Am J Physiol Regul Integr Comp Physiol, 284*(5), R1296-1305. Thompson, D. J., Molello, J. A., Strebing, R. J., & Dyke, I. L. (1978). Teratogenicity of adriamycin and daunomycin in the rat and rabbit. *Teratology, 17*(2), 151-157. Tsuchida, S., Matsusaka, T., Chen, X., Okubo, S., Niimura, F., Nishimura, H., Fogo, A.,

Utsunomiya, H., Inagami, T., & Ichikawa, I. (1998). Murine double nullizygotes of the angiotensin type 1A and 1B receptor genes duplicate severe abnormal

Obstruction) trial home page. October 12, 2011, Available from

infants with obstructive uropathy due to posterior urethral valves. *J Urol, 133*(2),

of congenital renal malformations by fetal ultrasonographic examination: an analysis of 709,030 births in 12 European countries. *Eur J Med Genet, 48*(2), 131-144.

contribution to end-stage renal disease in children. *Adv Ren Replace Ther, 8*(3), 157-163.

K. (2002). A disintegrin and metalloproteinase with thrombospondin motifs (ADAMTS)-1 null mutant mice develop renal lesions mimicking obstructive Anaemia is a common complication of chronic kidney disease (CKD). The underlying physiology related to anaemia in CKD is secondary to reduction in endogenous erythropoietin as the glomerular filtration rate (GFR) declines. The introduction of erythropoiesis stimulating agents (ESA) has revolutionized the management of anaemia in CKD, leading to substantial reductions in the blood transfusion requirements, improvement in energy and physical function and small improvements in health-related quality of life (Clement et. al., 2009; Eschbach et. al., 1987; Gandra et. al., 2010). Targeting higher haemoglobin with ESA therapy has been associated with increased risks of stroke, vascular access thrombosis, hypertension and possibly death (Badve et. al., 2011; Besarab et. al., 1998; Palmer et. al., 2010; Pfeffer et. al., 2009; Phrommintikul et. al., 2007; Singh et. al., 2006). The current KDOQI Clinical Practice Guideline recommends a haemoglobin target of 11-12g/dL. However, a substantial proportion of non-dialysis and dialysis CKD patients exhibit fluctuations in the haemoglobin levels, also known as haemoglobin variability. There is an emerging body of evidence demonstrating an association between haemoglobin variability and mortality in CKD patients treated with ESAs. Maintaining haemoglobin levels within narrow target range remains a major challenge in clinical practice. The aim of this chapter is to review the definition, prevalence, risk factors of haemoglobin variability, and its impact on survival, provide recommendations where possible and suggest directions for future research.
