History

Fact Explanation
Asymptomatic [1] [2] [3] Initial stages of CKD are asymptomatic and only a routine blood or urine investigation may point towards the diagnosis. [1] [2] [3]
Antenatal history of oligohydroamnios [4] The low volume of amniotic fluid during antenatal period suggests that the production of urine in the fetus is impaired and usually suggests a renal anomaly [4]
Respiratory difficulties, distended abdomen with weak musculature, and facial anomalies in a neontate [4] These are usually features of a syndrome called "Potter syndrome" due to bilateral renal agenesis and the lung hypoplasia causes respiratory difficulties. These neonates usually die soon after birth. [4]
Polyuria, Nocturia and Nocturnal eneuresis [5] Passage of large volumes of urine (Polyuria), Awakening at night to pass urine (Nocturia), and bed wetting usually suggest decrease in urine concentrating ability and could be an early sign of chronic kidney disease in children. [5]
History of febrile episodes with Frequency, urgency, dysuria, hesitancy, hematuria [5] These features usually suggest Urinary tract infection which can cause kidney damage in children and later progress to CKD if untreated. [5]
History of abdominal or flank pain [5] These features usually suggest acute pyelonephritis, urinary calculi, trauma to kidney or bladder which can cause CKD later [5]
Abdominal mass [5] It usually suggests hydronephrosis, multicystic, dysplastic or polycystic kidney disease, renal vein thrombosis, and Wilms tumor or neuroblastoma which can cause CKD [5]
Shortness of breath on exertion, easy fatiguability [5] [7] These features suggest anemia which occurs due to inadequate Erythropoietin production from the kidney which is needed for red blood cell synthesis [5] [7]
Ankle swelling [5] This is due to volume overload due to renal impairment. Reduced glomerular filtration of sodium, activation of the Renin-Angiotensin-Aldosterone system causes sodium and water retention [5] [6]
Anorexia and other gastrointestinal disturbances, bleeding [10] [13] Anorexia in CKD is due to decreased taste and smell of food, early satiety, dysfunctional hypothalamic membrane adenylate cyclase, increased brain tryptophan, and increased cytokine production. As well as GI bleeding is common and is thought to be due to increased association with angiodysplasia in these patients with CKD [10] [13]
Chest pain, shortness of breath on exertion [15] Atherosclerosis is common in CKD. This can cause myocardial ischemia and ischemic heart disease with angina, myocardial infarction, and sudden cardiac death. Additionally cardiomyopathy is also common due to pressure and volume overload. [15]
Shortness of breath, hemoptysis [9] Volume overload causes pulmonary edema which causes acute left ventricular failure and this causes dyspnea and blood stained sputum [9]
Headache, visual disturbances, altered level of consciousness [5] [6] [8] Hypertension is mainly due to voulme overload in renal impairment and hypertensive encephalopathy may lead to these symptoms Also uremic encephalopathy can lead to altered level of consciousness. [5] [8]
Itching of the body [11] Many factors contribute to itching in CKD. Skin of patients with chronic renal failure becomes atrophic and dry and pruritogenic cytokines may be produced in the dermis. As well as mast cells are high in number and this is thought to be due to the raised parathyroid hormone level due to secondary hyperparathyroidism.However in chronic dialysis patients with itching is due to increased level of calcium, magnesium and phosphate. Plasma histamine level is also higher in uraemic patients. Additionally there's an imbalance in the expression of the opioid receptor subtypes in uremia which can cause itching. [11]
Weight loss [10] anorexia leads to cachexia as well as protein calorie malnutrition is seen in these children [10]
Poor growth [12] This is multifactorial. The underlying disease, metabolic bone disease, acid base disturbances, anemia, protein-calorie malnutrition are some of contributing factors. Alterations is gonadotrophic and somatotrophic axis es are also seen [12]
Increased incidence of fractures [5] [7] Kidneys excrete phophorous and 1-α-hydroxylation of vitamin D. Inadequate 1, 25 dihydroxy-vitamin D levels cause serum calcium levels to fall in turn causing secondary hyperparathyroidism. Parathyroid hormone has a phosphaturic effect, as well as cause bone resorption and renal osteodystrophy results [5] [7]
References
  1. WHYTE D. A., FINE R. N.. Chronic Kidney Disease in Children. Pediatrics in Review [online] 2008 October, 29(10):335-341 [viewed 15 September 2014] Available from: doi:10.1542/pir.29-10-335
  2. QUIGLEY RAYMOND. Chronic Kidney Disease: Highlights for the General Pediatrician. International Journal of Pediatrics [online] 2012 December, 2012:1-5 [viewed 15 September 2014] Available from: doi:10.1155/2012/943904
  3. WARADY BRADLEY A., CHADHA VIMAL. Chronic kidney disease in children: the global perspective. Pediatr Nephrol [online] December, 22(12):1999-2009 [viewed 15 September 2014] Available from: doi:10.1007/s00467-006-0410-1
  4. TAGORE KOYYE RAVINDRANATH, RAMINENI ASOK KUMAR S., VIJAYA LAKSHMI A. R., N. BHAVANI. Prune Belly Syndrome. Case Reports in Pediatrics [online] 2011 December, 2011:1-3 [viewed 15 September 2014] Available from: doi:10.1155/2011/121736
  5. BARAKAT AMIN J.. Presentation of the Child with Renal Disease and Guidelines for Referral to the Pediatric Nephrologist. International Journal of Pediatrics [online] 2012 December, 2012:1-5 [viewed 15 September 2014] Available from: doi:10.1155/2012/978673
  6. HUNG SZU-CHUN, KUO KO-LIN, PENG CHING-HSIU, WU CHE-HSIUNG, LIEN YU-CHUNG, WANG YI-CHUN, TARNG DER-CHERNG. Volume overload correlates with cardiovascular risk factors in patients with chronic kidney disease. Kidney Int [online] December, 85(3):703-709 [viewed 15 September 2014] Available from: doi:10.1038/ki.2013.336
  7. THOMAS R, KANSO A, SEDOR JR. Chronic Kidney Disease and Its Complications Prim Care [online] 2008 Jun, 35(2):329-vii [viewed 15 September 2014] Available from: doi:10.1016/j.pop.2008.01.008
  8. TEDLA F. M., BRAR A., BROWNE R., BROWN C.. Hypertension in Chronic Kidney Disease: Navigating the Evidence. International Journal of Hypertension [online] 2011 December, 2011:1-9 [viewed 15 September 2014] Available from: doi:10.4061/2011/132405
  9. MANSOOR S.. 'Flash pulmonary oedema'--a diagnosis for both the cardiologist and the nephrologist?. [online] 2001 July, 16(7):1311-1313 [viewed 15 September 2014] Available from: doi:10.1093/ndt/16.7.1311
  10. CHEUNG WAI W., MAK ROBERT H.. Ghrelin in Chronic Kidney Disease. International Journal of Peptides [online] 2010 December, 2010:1-7 [viewed 15 September 2014] Available from: doi:10.1155/2010/567343
  11. TWYCROSS R.. Itch: scratching more than the surface. [online] 2003 January, 96(1):7-26 [viewed 15 September 2014] Available from: doi:10.1093/qjmed/hcg002
  12. SALAS PAULINA, PINTO VIOLA, RODRIGUEZ JOSEFINA, ZAMBRANO MARIA JOSE, MERICQ VERONICA. Growth Retardation in Children with Kidney Disease. International Journal of Endocrinology [online] 2013 December, 2013:1-8 [viewed 15 September 2014] Available from: doi:10.1155/2013/970946
  13. FABIAN G.. An unusual multiplex cause of severe gastrointestinal bleeding in a haemodialysed patient. [online] 2000 November, 15(11):1869-1871 [viewed 15 September 2014] Available from: doi:10.1093/ndt/15.11.1869
  14. HARAMBAT J, VAN STRALEN KJ, KIM JJ, TIZARD EJ. Epidemiology of chronic kidney disease in children Pediatr Nephrol [online] 2012 Mar, 27(3):363-373 [viewed 16 September 2014] Available from: doi:10.1007/s00467-011-1939-1
  15. SARNAK M. J.. Kidney Disease as a Risk Factor for Development of Cardiovascular Disease: A Statement From the American Heart Association Councils on Kidney in Cardiovascular Disease, High Blood Pressure Research, Clinical Cardiology, and Epidemiology and Prevention. Circulation [online] 2003 October, 108(17):2154-2169 [viewed 16 September 2014] Available from: doi:10.1161/01.CIR.0000095676.90936.80

Examination

Fact Explanation
No signs [1] [2] [3] Initial stages of CKD are asymptomatic and there are usually no signs on examination, only a routine blood or urine investigation may point towards the diagnosis. [1] [2] [3]
Charasteristic facies, protuberant abdomen [4] These are usually features of a syndrome called "Potter syndrome/ Prune belly syndrome" due to bilateral renal agenesis and the lung hypoplasia results. These neonates usually die soon after birth. [4]
Ear tags and ear anomalies [12] This is a feature of branchiootorenal (BOR) syndrome which is an autosomal dominant condition resulting in congenital abnormal development of the first and second branchial arches and urinary tract. Therefore ear anomalies are found in these children [12]
Pallor [5] [6] This suggests anemia which occurs due to inadequate Erythropoietin production from the kidney which is needed for red blood cell synthesis [5] [6]
Hyper pigmentation of the skin and other skin changes such as Xerosis/Icthyosis [13] This characteristic hyper pigmentation is due to an increase in melanin as a result of fai­lure of the kidneys to execrete B-melanocyte­stimulating hormone (B-MSH). [13]
Cachexia [7] anorexia leads to cachexia as well as protein calorie malnutrition is seen in these children [7]
Reduced growth parameters [8] This is multifactorial. The underlying disease, metabolic bone disease, acid base disturbances, anemia, protein-calorie malnutrition are some of contributing factors. Alterations is gonadotrophic and somatotrophic axis es are also seen [8]
Half and half nails./Leukonychia of nails [13] It's not clearly known but is thought due to melanin pigment in the nail plate. another theory postulated explains that it's due to the capillary density in the nail bed, and thickening of the capillary walls which forms the discolored band. Leukonychia is due to proteinuria in Nephrotic syndrome [13]
Arterio-venous fistula at the wrist [19] These children with chronic renal failure are usually on dialysis therefore iatrogenic A-V fistula may be seen [19]
Bilateral pitting ankle oedema [5] Reduced glomerular filtration of sodium, activation of the Renin-Angiotensin-Aldosterone system causes sodium and water retention and this volume overload causes edema. [5]
Scratch marks [9] Many factors contribute to itching in CKD. Skin of patients with chronic renal failure becomes atrophic and dry and pruritogenic cytokines may be produced in the dermis. As well as mast cells are high in number and this is thought to be due to the raised parathyroid hormone level due to secondary hyperparathyroidism.However in chronic dialysis patients with itching is due to increased level of calcium, magnesium and phosphate. Plasma histamine level is also higher in uraemic patients. Additionally there's an imbalance in the expression of the opioid receptor subtypes in uremia which can cause itching [9]
Elevated Jugular Venous Pressure [17] Volume overload and ultimate cardiac failure both cause JVP to be elevated [17]
Elevated blood pressure [10] Volume overload causes hypertension [10]
Cardiomegaly [16] Heart failure causes cardiomegaly. Cardiomegaly is due to increased work load due to volume overload [16]
Gallop rhythm [18] Heart failure due to volume overload causes gallop rhythm [18]
Bilateral end inspiratory crepitations [11] Volume overload causes heart failure and pulmonary edema due to venous congestion in the lungs cause bilateral end inspiratory crepitations [11]
Ballotable kidneys [5] It usually suggests hydronephrosis, multicystic, dysplastic or polycystic kidney disease, renal vein thrombosis, and Wilms tumor or neuroblastoma which can cause CKD [5]
Altered level of consciousness, Confusion, Coma [14] This is usually due to uremic encephalopathy but hypertensive encephalopathy too can cause this [14]
Bowing of legs, widening of the wrists, frontal bossing, Rachitic rossary [15] These are features of rickets due to inadequate active form of vitamin D due to renal impairment. Therefore characteristic skeletal changes and clinical features of Rickets appear [15]
Tenderness and stiffness in joints [20] This is due to metabolic bone disease in these children. It can also produce spontaneous tendon rupture, predisposition to fracture, and proximal muscle weakness. [20]
References
  1. WHYTE D. A., FINE R. N.. Chronic Kidney Disease in Children. Pediatrics in Review [online] 2008 October, 29(10):335-341 [viewed 15 September 2014] Available from: doi:10.1542/pir.29-10-335
  2. QUIGLEY RAYMOND. Chronic Kidney Disease: Highlights for the General Pediatrician. International Journal of Pediatrics [online] 2012 December, 2012:1-5 [viewed 15 September 2014] Available from: doi:10.1155/2012/943904
  3. WARADY BRADLEY A., CHADHA VIMAL. Chronic kidney disease in children: the global perspective. Pediatr Nephrol [online] December, 22(12):1999-2009 [viewed 15 September 2014] Available from: doi:10.1007/s00467-006-0410-1
  4. TAGORE KOYYE RAVINDRANATH, RAMINENI ASOK KUMAR S., VIJAYA LAKSHMI A. R., N. BHAVANI. Prune Belly Syndrome. Case Reports in Pediatrics [online] 2011 December, 2011:1-3 [viewed 15 September 2014] Available from: doi:10.1155/2011/121736
  5. BARAKAT AMIN J.. Presentation of the Child with Renal Disease and Guidelines for Referral to the Pediatric Nephrologist. International Journal of Pediatrics [online] 2012 December, 2012:1-5 [viewed 15 September 2014] Available from: doi:10.1155/2012/978673
  6. THOMAS R, KANSO A, SEDOR JR. Chronic Kidney Disease and Its Complications Prim Care [online] 2008 Jun, 35(2):329-vii [viewed 15 September 2014] Available from: doi:10.1016/j.pop.2008.01.008
  7. CHEUNG WAI W., MAK ROBERT H.. Ghrelin in Chronic Kidney Disease. International Journal of Peptides [online] 2010 December, 2010:1-7 [viewed 15 September 2014] Available from: doi:10.1155/2010/567343
  8. SALAS PAULINA, PINTO VIOLA, RODRIGUEZ JOSEFINA, ZAMBRANO MARIA JOSE, MERICQ VERONICA. Growth Retardation in Children with Kidney Disease. International Journal of Endocrinology [online] 2013 December, 2013:1-8 [viewed 15 September 2014] Available from: doi:10.1155/2013/970946
  9. TWYCROSS R.. Itch: scratching more than the surface. [online] 2003 January, 96(1):7-26 [viewed 15 September 2014] Available from: doi:10.1093/qjmed/hcg002
  10. TEDLA F. M., BRAR A., BROWNE R., BROWN C.. Hypertension in Chronic Kidney Disease: Navigating the Evidence. International Journal of Hypertension [online] 2011 December, 2011:1-9 [viewed 15 September 2014] Available from: doi:10.4061/2011/132405
  11. MANSOOR S.. 'Flash pulmonary oedema'--a diagnosis for both the cardiologist and the nephrologist?. [online] 2001 July, 16(7):1311-1313 [viewed 15 September 2014] Available from: doi:10.1093/ndt/16.7.1311
  12. SCHIFF M.. Ear and kidney malformations with renal failure in an infant: what is the link?. Nephrology Dialysis Transplantation [online] 2003 August, 18(8):1673-1674 [viewed 15 September 2014] Available from: doi:10.1093/ndt/gfg235
  13. FALODUN O, OGUNBIYI A, SALAKO B, GEORGE AK. Skin changes in patients with chronic renal failure. Saudi J Kidney Dis Transpl [online] 2011 Mar, 22(2):268-72 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21422624
  14. MOODALBAIL D. G., REISER K. A., DETRE J. A., SCHULTZ R. T., HERRINGTON J. D., DAVATZIKOS C., DOSHI J. J., ERUS G., LIU H.-S., RADCLIFFE J., FURTH S. L., HOOPER S. R.. Systematic Review of Structural and Functional Neuroimaging Findings in Children and Adults with CKD. Clinical Journal of the American Society of Nephrology [online] December, 8(8):1429-1448 [viewed 15 September 2014] Available from: doi:10.2215/​CJN.11601112
  15. HOLICK MF. Resurrection of vitamin D deficiency and rickets J Clin Invest [online] 2006 Aug 1, 116(8):2062-2072 [viewed 15 September 2014] Available from: doi:10.1172/JCI29449
  16. GLASSOCK R. J., PECOITS-FILHO R., BARBERATO S. H.. Left Ventricular Mass in Chronic Kidney Disease and ESRD. Clinical Journal of the American Society of Nephrology [online] December, 4(Supplement 1):S79-S91 [viewed 15 September 2014] Available from: doi:10.2215/​CJN.04860709
  17. AHMED A. DEFEAT - Heart Failure: A Guide to Management of Geriatric Heart Failure by Generalist Physicians Minerva Med [online] 2009 Feb, 100(1):39-50 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2914573
  18. GRAYZEL J.. Gallop Rhythm of the Heart: II. Quadruple Rhythm and its Relation to Summation and Augmented Gallops. Circulation [online] 1959 December, 20(6):1053-1062 [viewed 15 September 2014] Available from: doi:10.1161/01.CIR.20.6.1053
  19. HIREMATH SWAPNIL, KNOLL GREG, WEINSTEIN MILTON C., SINGH SHREE RAM. Should the Arteriovenous Fistula Be Created before Starting Dialysis?: A Decision Analytic Approach. PLoS ONE [online] 2011 December [viewed 15 September 2014] Available from: doi:10.1371/journal.pone.0028453
  20. MARTIN K. J., GONZALEZ E. A.. Metabolic Bone Disease in Chronic Kidney Disease. Journal of the American Society of Nephrology [online] 2007 January, 18(3):875-885 [viewed 16 September 2014] Available from: doi:10.1681/ASN.2006070771

Differential Diagnoses

Fact Explanation
Acute kidney injury [6] Sometimes acute kidney injury maybe confused with CKD with the presentation and metabolic anomalies. But this is more sudden onset and progress over a short period of time. [6]
Acute on chronic kidney disease [1] Sometimes a patient may present with features of acute kidney injury in the back ground of chronic kidney disease. [1]
Urinary tract infection [5] Recurrent untreated urinary tract infections can result in significant kidney damage in developing kidneys and children and subsequent CKD [5]
Hereditary nephropathies [3] [4] Among the hereditary nephropathies, polycystic kidney disease and Alport syndrome are common and cause CKD in later life [3] [4]
Systemic diseases causing CKD [7] [8] Systemic diseases such as diabetic nephropathy, lupus nephritis can cause CKD in long term [7] [8]
Congestive cardiac failure [9] Sometimes the clinical presentation may be confused with a child presenting with heart failure with bilateral ankle edema, cardiomegaly and other features of heart failure. Alternatively CKD can lead to heart failure as well. [9]
Chronic liver cell disease (CLCD) [10] A child with CLCD can present with ankle swelling, and maybe confused with CKD [10]
References
  1. BELLOMO RINALDO, RONCO CLAUDIO, KELLUM JOHN A, MEHTA RAVINDRA L, PALEVSKY PAUL. . Crit Care [online] 2004 December [viewed 16 September 2014] Available from: doi:10.1186/cc2872
  2. KASHTAN C. E.. Alport syndrome and the X chromosome: implications of a diagnosis of Alport syndrome in females. Nephrology Dialysis Transplantation [online] 2007 March, 22(6):1499-1505 [viewed 15 September 2014] Available from: doi:10.1093/ndt/gfm024
  3. BARAKAT AMIN J.. Presentation of the Child with Renal Disease and Guidelines for Referral to the Pediatric Nephrologist. International Journal of Pediatrics [online] 2012 December, 2012:1-5 [viewed 15 September 2014] Available from: doi:10.1155/2012/978673
  4. HABIB SABEEN. Highlights for Management of a Child with a Urinary Tract Infection. International Journal of Pediatrics [online] 2012 December, 2012:1-6 [viewed 15 September 2014] Available from: doi:10.1155/2012/943653
  5. ESEZOBOR CHRISTOPHER IMOKHUEDE, LADAPO TAIWO AUGUSTINA, OSINAIKE BABAYEMI, LESI FOLUSO EBUN AFOLABI, SEGURO ANTONIO CARLOS. Paediatric Acute Kidney Injury in a Tertiary Hospital in Nigeria: Prevalence, Causes and Mortality Rate. PLoS ONE [online] 2012 December [viewed 15 September 2014] Available from: doi:10.1371/journal.pone.0051229
  6. RAILE K., GALLER A., HOFER S., HERBST A., DUNSTHEIMER D., BUSCH P., HOLL R. W.. Diabetic Nephropathy in 27,805 Children, Adolescents, and Adults With Type 1 Diabetes: Effect of diabetes duration, A1C, hypertension, dyslipidemia, diabetes onset, and sex. Diabetes Care [online] 2007 July, 30(10):2523-2528 [viewed 15 September 2014] Available from: doi:10.2337/dc07-0282
  7. RUGGIERO B., VIVARELLI M., GIANVITI A., BENETTI E., PERUZZI L., BARBANO G., CORONA F., VENTURA G., PECORARO C., MURER L., GHIGGERI G. M., PENNESI M., EDEFONTI A., COPPO R., EMMA F.. Lupus nephritis in children and adolescents: results of the Italian Collaborative Study. Nephrology Dialysis Transplantation [online] December, 28(6):1487-1496 [viewed 15 September 2014] Available from: doi:10.1093/ndt/gfs589
  8. HSU D. T., PEARSON G. D.. Heart Failure in Children: Part I: History, Etiology, and Pathophysiology. Circulation: Heart Failure [online] 2009 January, 2(1):63-70 [viewed 15 September 2014] Available from: doi:10.1161/CIRCHEARTFAILURE.108.820217
  9. KASHANI A., LANDAVERDE C., MEDICI V., ROSSARO L.. Fluid retention in cirrhosis: pathophysiology and management. QJM [online] 2008 January, 101(2):71-85 [viewed 15 September 2014] Available from: doi:10.1093/qjmed/hcm121

Investigations - for Diagnosis

Fact Explanation
Full blood count and blood picture [1] Low Hemoglobin is seen in CKD and blood picture may show normochromic normocytic anemia [1]
Blood urea nitrogen [2] This is elevated and this is due to reduced excretory capacity of the kidneys. [2]
Serum creatinine [12] This is elevated and this is due to reduced excretory capacity of the kidneys. [12]
Serum Sodium/ Potassium/ Calcium/ Magnesium/ Phophorous [1] [3] Serum electrolytes are important as electrolyte imbalances are common with high Potassium, Low Sodium, Calcium and Magnesium and high phosphorous. [1] [3]
Serum Alkaline Phosphatase (ALP) [4] With secondary hyper parathyroidsm, ALP is elevated [4]
Serum Parathyroid hormone [4] This is elevated due to secondary hyper parathyroidsm [4]
Serum cholesterol [1] Blood lipid levels are elevated in CKD [1]
Estimated glomerular filtration rate [1] This is the most important investigation to diagnose and stage the disease. This uses serum creatinine concentration, either the Cockcroft-Gault or the Modification of Diet in Renal Disease (MDRD) Study estimating equations [1]
Arterial blood gas analysis [7] Acid base disturbances with metabolic acidosis is seen in CKD therefore this is important [7]
Urine full report [5] This may show proteins, red blood cells, white blood cells, red cell casts aid in making a diagnosis [5]
Urine for microalbuminuria [6] This identifies the earliest abnormality that is filtration of microalbumin before manifestation of proteinuria [6]
Ultrasound kidney-ureter-bladder [8] It's cheap. widely available and easy to use and chronic renal parenchymal changes can be identified as well as etiology such as ureteric stones, congenital anomalies [8]
Radionuclide studies [9] [10] DMSA, MCUG scans are important in determining scarring in CKD [9] [10]
Skeletal x-rays [1] The changes seen are osteitis fibrosa cystica, osteomalacia, adynamic bone disorder and mixed osteodystrophy. This is important in ruling out the presence of changes due to secondary hyper parathyroidism [1]
Renal biopsy [11] This is mainly to identify the etiology but will not be of use in end stage renal disease in which the whole kidney is scarred [11]
References
  1. THOMAS R, KANSO A, SEDOR JR. Chronic Kidney Disease and Its Complications Prim Care [online] 2008 Jun, 35(2):329-vii [viewed 15 September 2014] Available from: doi:10.1016/j.pop.2008.01.008
  2. MARTIN WILLIAM F, ARMSTRONG LAWRENCE E, RODRIGUEZ NANCY R. . Nutr Metab (Lond) [online] 2005 December [viewed 15 September 2014] Available from: doi:10.1186/1743-7075-2-25
  3. KORGAONKAR S., TILEA A., GILLESPIE B. W., KISER M., EISELE G., FINKELSTEIN F., KOTANKO P., PITT B., SARAN R.. Serum Potassium and Outcomes in CKD: Insights from the RRI-CKD Cohort Study. Clinical Journal of the American Society of Nephrology [online] December, 5(5):762-769 [viewed 15 September 2014] Available from: doi:10.2215/​CJN.05850809
  4. KOVESDY C. P., KALANTAR-ZADEH K.. Diuretics and secondary hyperparathyroidism in chronic kidney disease. Nephrology Dialysis Transplantation [online] December, 26(4):1122-1125 [viewed 15 September 2014] Available from: doi:10.1093/ndt/gfr083
  5. FRASER SIMON D. S., RODERICK PAUL J., MCINTYRE NATASHA J., HARRIS SCOTT, MCINTYRE CHRISTOPHER, FLUCK RICHARD, TAAL MAARTEN W., REMUZZI GIUSEPPE. Assessment of Proteinuria in Patients with Chronic Kidney Disease Stage 3: Albuminuria and Non-Albumin Proteinuria. PLoS ONE [online] 2014 May [viewed 15 September 2014] Available from: doi:10.1371/journal.pone.0098261
  6. SARAFIDIS P. A.. Microalbuminuria and chronic kidney disease as risk factors for cardiovascular disease. Nephrology Dialysis Transplantation [online] 2006 September, 21(9):2366-2374 [viewed 15 September 2014] Available from: doi:10.1093/ndt/gfl309
  7. CHEN WEI, ABRAMOWITZ MATTHEW K. Metabolic acidosis and the progression of chronic kidney disease. Array [online] 2014 December [viewed 15 September 2014] Available from: doi:10.1186/1471-2369-15-55
  8. HAMMAD ATHER M, JAFRI AFTAB H, NASIR SULAIMAN M. . BMC Med Imaging [online] 2004 December [viewed 15 September 2014] Available from: doi:10.1186/1471-2342-4-2
  9. BRAKEMAN PAUL. Vesicoureteral Reflux, Reflux Nephropathy, and End-Stage Renal Disease. Advances in Urology [online] 2008 December, 2008:1-7 [viewed 15 September 2014] Available from: doi:10.1155/2008/508949
  10. PRASAD MICHAELLA M., CHENG EARL Y.. Radiographic Evaluation of Children with Febrile Urinary Tract Infection: Bottom-Up, Top-Down, or None of the Above?. Advances in Urology [online] 2012 December, 2012:1-8 [viewed 15 September 2014] Available from: doi:10.1155/2012/716739
  11. HAIDER DOMINIK G, FRIEDL ALEXANDER, PERIC SLOBODAN, WIESINGER GüNTHER F, WOLZT MICHAEL, PROSENZ JULIAN, FISCHER HENRIK, HöRL WALTER H, SOLEIMAN AFSCHIN, FUHRMANN VALENTIN. Kidney biopsy in patients with glomerulonephritis: is the earlier the better?. Array [online] 2012 December [viewed 15 September 2014] Available from: doi:10.1186/1471-2369-13-34
  12. WALKER A. M.. Anemia as a Predictor of Cardiovascular Events in Patients with Elevated Serum Creatinine. Journal of the American Society of Nephrology [online] 2006 August, 17(8):2293-2298 [viewed 15 September 2014] Available from: doi:10.1681/ASN.2005020183

Investigations - Fitness for Management

Fact Explanation
Full blood count [1] [2] [4] To exclude any anemia and low platelet count prior to surgery (Renal transplant). Low platelet count maybe observed in iron repletion therapy [1] [2] [4]
Coagulation studies [1] [2] To exclude any coagulopathy before surgical management [1] [2]
Renal function tests- Serum creatinine, Blood urea nitrogen [1] [2] As these patients have impaired renal function, it's important to determine the severity prior to anesthesia [1] [2]
HLA typing [3] This is important to be done prior to renal transplant as HLA should be matched between donor and recipient for a better outcome [3]
References
  1. KUMAR A, SRIVASTAVA U. Role of routine laboratory investigations in preoperative evaluation J Anaesthesiol Clin Pharmacol [online] 2011, 27(2):174-179 [viewed 15 September 2014] Available from: doi:10.4103/0970-9185.81824
  2. SHULMAN M. A., THOMPSON B. R.. I. Not fit for a haircut ... how should we assess fitness and stratify risk for surgery?. British Journal of Anaesthesia [online] December, 112(6):955-957 [viewed 15 September 2014] Available from: doi:10.1093/bja/aeu003
  3. MAHDI BATOOL MUTAR. A glow of HLA typing in organ transplantation. Array [online] 2013 December [viewed 15 September 2014] Available from: doi:10.1186/2001-1326-2-6
  4. YESSAYAN LENAR, YEE JERRY, ZASUWA GARY, FRINAK STAN, BESARAB ANATOLE. Iron repletion is associated with reduction in platelet counts in non-dialysis chronic kidney disease patients independent of erythropoiesis-stimulating agent use: a retrospective cohort study. Array [online] 2014 December [viewed 16 September 2014] Available from: doi:10.1186/1471-2369-15-119

Investigations - Followup

Fact Explanation
Full blood count and blood picture [1] [7] Low Hemoglobin is seen in CKD and Low platelet count maybe observed with iron repletion therapy. Blood picture may show normochromic normocytic anemia and this is important in follow up of the patient with treatment [1] [7]
Blood urea nitrogen [4] Reduced excretory capacity of the kidneys causes elevation and usually done in follow up. [4]
Serum creatinine [2] Reduced excretory capacity of the kidneys causes elevation and usually done in follow up. [2]
Serum Sodium/ Potassium/ Calcium/ Magnesium/ Phophorous [1] [3] Serum electrolytes are important as electrolyte imbalances are common with high Potassium, Low Sodium, Calcium and Magnesium and high phosphorous. [1] [3]
Serum cholesterol [1] Blood lipid levels are elevated in CKD and done in the follow up [1]
Estimated glomerular filtration rate [1] This is the most important investigation to diagnose and stage the disease. This uses serum creatinine concentration, either the Cockcroft-Gault or the Modification of Diet in Renal Disease (MDRD) Study estimating equations [1]
Skeletal x-rays [1] As renal osteodystrophy occurs inadequate active form of Vitamin D production, rickets changes, osteopenia, osteoprorois can be identified. [1]
2D echocardiography [1] As these patients have high cardiovascular risk due to pressure overload due to long standing hypertension it's important to do a cardiac echo and look for left ventricular hypertrophy [1]
Blood pressure [5] As hypertension develops as a complication, it's important to monitor blood pressure long term [5]
Growth parameters [6] As growth impairment is seen in these children, it's important to monitor height and weight during follow up [6]
References
  1. THOMAS R, KANSO A, SEDOR JR. Chronic Kidney Disease and Its Complications Prim Care [online] 2008 Jun, 35(2):329-vii [viewed 15 September 2014] Available from: doi:10.1016/j.pop.2008.01.008
  2. WALKER A. M.. Anemia as a Predictor of Cardiovascular Events in Patients with Elevated Serum Creatinine. Journal of the American Society of Nephrology [online] 2006 August, 17(8):2293-2298 [viewed 15 September 2014] Available from: doi:10.1681/ASN.2005020183
  3. KORGAONKAR S., TILEA A., GILLESPIE B. W., KISER M., EISELE G., FINKELSTEIN F., KOTANKO P., PITT B., SARAN R.. Serum Potassium and Outcomes in CKD: Insights from the RRI-CKD Cohort Study. Clinical Journal of the American Society of Nephrology [online] December, 5(5):762-769 [viewed 15 September 2014] Available from: doi:10.2215/CJN.05850809
  4. MARTIN WILLIAM F, ARMSTRONG LAWRENCE E, RODRIGUEZ NANCY R. . Nutr Metab (Lond) [online] 2005 December [viewed 15 September 2014] Available from: doi:10.1186/1743-7075-2-25
  5. TEDLA F. M., BRAR A., BROWNE R., BROWN C.. Hypertension in Chronic Kidney Disease: Navigating the Evidence. International Journal of Hypertension [online] 2011 December, 2011:1-9 [viewed 15 September 2014] Available from: doi:10.4061/2011/132405
  6. SALAS PAULINA, PINTO VIOLA, RODRIGUEZ JOSEFINA, ZAMBRANO MARIA JOSE, MERICQ VERONICA. Growth Retardation in Children with Kidney Disease. International Journal of Endocrinology [online] 2013 December, 2013:1-8 [viewed 15 September 2014] Available from: doi:10.1155/2013/970946
  7. YESSAYAN LENAR, YEE JERRY, ZASUWA GARY, FRINAK STAN, BESARAB ANATOLE. Iron repletion is associated with reduction in platelet counts in non-dialysis chronic kidney disease patients independent of erythropoiesis-stimulating agent use: a retrospective cohort study. Array [online] 2014 December [viewed 16 September 2014] Available from: doi:10.1186/1471-2369-15-119

Investigations - Screening/Staging

Fact Explanation
Estimated glomerular filtration rate [1] This is the most important investigation to diagnose and stage the disease. This uses serum creatinine concentration, either the Cockcroft-Gault or the Modification of Diet in Renal Disease (MDRD) Study estimating equations. The staging is done as below. Stage 1-normal eGFR ≥ 90 mL/min per 1.73 m2 and persistent albuminuria, Stage 2-eGFR between 60 to 89 mL/min per 1.73 m2, Stage 3- eGFR between 30 to 59 mL/min per 1.73 m2, Stage 4- eGFR between 15 to 29 mL/min per 1.73 m2, Stage 5- eGFR of < 15 mL/min per 1.73 m2 or end-stage renal disease [1]
Urine full report for protein [2] This might help to detect albuminuria and this is thought to be associated with high cardiovascular risk. [2]
Urine dipstick for protein [3] Urine dipstick for protein may help to identify early CKD and can be done as mass screening [3]
Antenatal ultrasound scan [4] The low volume of amniotic fluid during antenatal period suggests that the production of urine in the fetus is impaired and usually suggests a renal anomaly and other congenital anomalies can be identified [4]
References
  1. THOMAS R, KANSO A, SEDOR JR. Chronic Kidney Disease and Its Complications Prim Care [online] 2008 Jun, 35(2):329-vii [viewed 15 September 2014] Available from: doi:10.1016/j.pop.2008.01.008
  2. GANSEVOORT R. T., DE JONG P. E.. The Case for Using Albuminuria in Staging Chronic Kidney Disease. Journal of the American Society of Nephrology [online] 2009 February, 20(3):465-468 [viewed 15 September 2014] Available from: doi:10.1681/ASN.2008111212
  3. HARAMBAT J, VAN STRALEN KJ, KIM JJ, TIZARD EJ. Epidemiology of chronic kidney disease in children Pediatr Nephrol [online] 2012 Mar, 27(3):363-373 [viewed 15 September 2014] Available from: doi:10.1007/s00467-011-1939-1
  4. TAGORE KOYYE RAVINDRANATH, RAMINENI ASOK KUMAR S., VIJAYA LAKSHMI A. R., N. BHAVANI. Prune Belly Syndrome. Case Reports in Pediatrics [online] 2011 December, 2011:1-3 [viewed 15 September 2014] Available from: doi:10.1155/2011/121736

Management - General Measures

Fact Explanation
Patient and parental education [7] This is a chronic disease, therefore patient education plays a major role. The parents are educated on etiology, nature, course, prognosis, available treatment options and importance of follow up. It's important to improve their quality of life. [7]
Diet and nutrition [5] [6] Anorexia and malnutrition is common , therefore calorie supplementation is necessary. Severe anorexia may indicate nasogastric or gastrostomy feeding. Protein intake should be sufficient. Phosphorous containing food such as milk products, Potassium containing fruits should be restricted. Salt restriction may be necessary. [5] [6]
Acute management of hyperkalemia [1] When the child presents with hyperkalemia, management is crucial as it can cause cardiac arrest if not treated. Ca gluconate is given if there are any ECG changes followed by insulin with glucose, intravenous or nebulized salbutamol. Sodium polystyrene sulphonate which is a gut potassium binding resin can be given as well. Dialysis is the definitive management. [1]
Acute management of metabolic acidosis [2] Intravenous bicarbonate may be necessary [2]
Acute management of acute pulmonary edema [3] High flow oxygen followed with intravenous Furosemide, Nitrates are used. [3]
Management of anemia [4] Oral and intravenous iron supplementation is done. But recombinant erythropoietin is usually necessary. The target Hemoglobin level is 11-12 g/dl. [4]
Management of bone disease [5] Gut phosphate binders are used to reduce phosphate and these are either calcium or aluminum based. But calcium-free, phosphate have been developed, such as the nonabsorbable agent sevelamer which neither has calcium or aluminum. Vitamin D is given as well as calcimimetics, agents which increase the calcium sensitivity of the calcium receptor in the parathyroid gland, down-regulating parathyroid hormone secretion and reducing hyperplasia of the parathyroid gland [5]
Management of hypertension [5] According to KDOQI guidelines target blood pressure is less than 130/85 mm Hg for all patients with kidney disease and less than 125/75 mmHg for patients with urinary protein excretion greater than 1g/24h. Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers, are the first-line agents [5]
Management of hyperlipidemia [5] Life style changes and Drugs may be used as Statins according to blood cholesterol level. [5]
Management of hormonal abnormalities [6] Recombinant human growth hormone is effective as well as safe for the treatment of growth failure in CKD children along with good nutrition [6]
Patient identification [8] These patients should be provided with a diagnosis card stating that they are having CKD. This is important especially when they go to other doctors and seek medication for other illnesses because certain drugs are contraindicated in CKD [8]
References
  1. AHEE P.. The management of hyperkalaemia in the emergency department. [online] 2000 May, 17(3):188-191 [viewed 15 September 2014] Available from: doi:10.1136/emj.17.3.188
  2. MACCARI C.. The patient with a severe degree of metabolic acidosis: a deductive analysis. QJM [online] 2006 March, 99(7):475-485 [viewed 15 September 2014] Available from: doi:10.1093/qjmed/hcl069
  3. MANSOOR S.. 'Flash pulmonary oedema'--a diagnosis for both the cardiologist and the nephrologist?. [online] 2001 July, 16(7):1311-1313 [viewed 15 September 2014] Available from: doi:10.1093/ndt/16.7.1311
  4. LEWIS M., SHAW J., REID C., EVANS J., WEBB N., VERRIER-JONES K.. Aspects of anaemia management in children with established renal failure (Chapter 15). Nephrology Dialysis Transplantation [online] 2007 August, 22(Supplement 7):vii181-vii183 [viewed 15 September 2014] Available from: doi:10.1093/ndt/gfm338
  5. THOMAS R, KANSO A, SEDOR JR. Chronic Kidney Disease and Its Complications Prim Care [online] 2008 Jun, 35(2):329-vii [viewed 15 September 2014] Available from: doi:10.1016/j.pop.2008.01.008
  6. SALAS PAULINA, PINTO VIOLA, RODRIGUEZ JOSEFINA, ZAMBRANO MARIA JOSE, MERICQ VERONICA. Growth Retardation in Children with Kidney Disease. International Journal of Endocrinology [online] 2013 December, 2013:1-8 [viewed 15 September 2014] Available from: doi:10.1155/2013/970946
  7. GERSON A. C., WENTZ A., ABRAHAM A. G., MENDLEY S. R., HOOPER S. R., BUTLER R. W., GIPSON D. S., LANDE M. B., SHINNAR S., MOXEY-MIMS M. M., WARADY B. A., FURTH S. L.. Health-Related Quality of Life of Children With Mild to Moderate Chronic Kidney Disease. PEDIATRICS [online] December, 125(2):e349-e357 [viewed 15 September 2014] Available from: doi:10.1542/peds.2009-0085
  8. PERAZELLA M. A.. Renal Vulnerability to Drug Toxicity. Clinical Journal of the American Society of Nephrology [online] December, 4(7):1275-1283 [viewed 15 September 2014] Available from: doi:10.2215/​CJN.02050309

Management - Specific Treatments

Fact Explanation
Pharmacological therapy for the underlying disease [3] [8] If the underlying cause is an auto immune disease such as Lupus nephritis management with immunosupressants are necessary such as steroids. Alternatively recurrent Urinary tract infections should be treated with proper antibiotic therapy [3] [8]
Surgical interventions for the underlying disease [4] If the underlying cause is obstructive uropathy, congenital anomalies corrective surgery is necessary to reverse the course [4]
Dialysis [1] [2] This is a form of renal replacement therapy. Usually dialysis is indicated when the glomerular filtration rate (GFR) is <15 mL/min and there is one or more of the following such as symptoms or signs of uraemia, inability to control hydration status or blood pressure or a progressive deterioration in nutritional status. Dialysis should be started before the GFR has fallen to 6 mL/min/1.73m2 even if asymptomatic. Hemodialysis is commonly done [1] [2]
Renal transplantation [5] [6] [7] This is the ultimate management option as a form of replacement therapy. But finding a matched donor is crucial and following the transplantation, complications can occur. The child is usually on immunosuppressants life long. [5] [6] [7]
References
  1. TATTERSALL J., DEKKER F., HEIMBURGER O., JAGER K. J., LAMEIRE N., LINDLEY E., VAN BIESEN W., VANHOLDER R., ZOCCALI C.. When to start dialysis: updated guidance following publication of the Initiating Dialysis Early and Late (IDEAL) study. Nephrology Dialysis Transplantation [online] December, 26(7):2082-2086 [viewed 15 September 2014] Available from: doi:10.1093/ndt/gfr168
  2. ROSANSKY S., GLASSOCK R. J., CLARK W. F.. Early Start of Dialysis: A Critical Review. Clinical Journal of the American Society of Nephrology [online] December, 6(5):1222-1228 [viewed 15 September 2014] Available from: doi:10.2215/​CJN.09301010
  3. APPEL G. B.. New and future therapies for lupus nephritis. Cleveland Clinic Journal of Medicine [online] December, 79(2):134-140 [viewed 15 September 2014] Available from: doi:10.3949/ccjm.78gr.11004
  4. VAKALOPOULOS IOANNIS, KAMPANTAIS SPYRIDON, DIMOPOULOS PANAGIOTIS, PAPASTAVROS CHRISTOS, KATSIKAS VASILEIOS. Treatment of obstructive uropathy in one of three young brothers suffering from Gorlin-Cohen syndrome: a case report. Array [online] 2012 December [viewed 15 September 2014] Available from: doi:10.1186/1471-2490-12-2
  5. SAMUEL S. M., TONELLI M. A., FOSTER B. J., ALEXANDER R. T., NETTEL-AGUIRRE A., SOO A., HEMMELGARN B. R.. Survival in Pediatric Dialysis and Transplant Patients. Clinical Journal of the American Society of Nephrology [online] December, 6(5):1094-1099 [viewed 15 September 2014] Available from: doi:10.2215/​CJN.04920610
  6. DONATI-BOURNE J., ROBERTS H. W., COLEMAN R. A.. Donor-Recipient Size Mismatch in Paediatric Renal Transplantation. Journal of Transplantation [online] 2014 December, 2014:1-5 [viewed 15 September 2014] Available from: doi:10.1155/2014/317574
  7. KRAMER A., STEL V. S., GESKUS R. B., TIZARD E. J., VERRINA E., SCHAEFER F., HEAF J. G., KRAMAR R., KRISCHOCK L., LEIVESTAD T., PALSSON R., RAVANI P., JAGER K. J.. The effect of timing of the first kidney transplantation on survival in children initiating renal replacement therapy. Nephrology Dialysis Transplantation [online] December, 27(3):1256-1264 [viewed 15 September 2014] Available from: doi:10.1093/ndt/gfr493
  8. HABIB SABEEN. Highlights for Management of a Child with a Urinary Tract Infection. International Journal of Pediatrics [online] 2012 December, 2012:1-6 [viewed 15 September 2014] Available from: doi:10.1155/2012/943653