| 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] |
| 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] |
| 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] |
| 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] |
| 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] |
| 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] |
| 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] |
| 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] |
| 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] |