2. Case scenario
Hasan, a 4 years old boy, immunized, hailing from tangail came
with the complaints of polyuria, polydipsia for 4 months, failure
to thrive for last 3 months.
On query, irregular fever for 2 months, there was no history of
contact with TB patient & loss of appetite.
O/E- he was moderately pale, hypertensive with growth retard.
No organomegaly.
4. Introduction
Chronic kidney disease is a chronic progressive disease that
can hamper normal lifestyle and can shorten lifespan of an
individual.
Today we try to focus on various aspects of CKD
• Definition
• Etiology of CKD
• Pathophysiology and pathogenesis
• Stages of CKD
• Clinical features
• Approach to investigation
• Management
5. Definition of Chronic Kidney Disease
(NKF KDOQI Guidelines)
1. Kidney damage for ≥3 months, with or without decreased GFR,
manifested by 1 or more of the following features:
• Abnormalities in the composition of the blood or urine
• Abnormalities in imaging tests
• Abnormalities on kidney biopsy
2. GFR <60 mL/min/1.73 m2 for ≥3 months, with or without the
other signs of kidney damage described above.
6. • The above definition is not applicable to children
younger than 2 years, because they normally have a
low GFR, even when corrected for body surface area.
• In these patients, calculated GFR based on serum
creatinine can be compared with normative age-
appropriate values to detect renal impairment.
7. Glomerular filtration rate
Modified Schwartz formula:
K × Height in cm
Serum creatinine in mg/dL
eGFR(mL/min/1.73m²)=
• Here k= 0.43.
• Can be used for GFR between 15-75 ml/min/1.73m².
• In children between 1-16yr.
8. Prevalence
• Globally, The prevalence of CKD in the pediatric
population is approximately 18 per 1 million.
• Among children, chronic kidney disease is more
common in children older than 6 years.
9. Etiology
CKD in children <5 year old
Most common congenital abnormalities
• renal hypoplasia, dysplasia or obstructive uropathy.
Additional causes
• congenital nephrotic syndrome
• prune belly syndrome, cortical necrosis
• focal segmental glomerulosclerosis
• autosomal recessive polycystic kidney disease
• renal vein thrombosis and HUS.
10. After 5 year of age
Acquired diseases
• lupus nephritis
Inherited disorders
• familial juvenile nephronophthisis
• Alport syndrome.
For all age group
• Metabolic disorders : Cystinosis, Hyperoxaluria.
• Inherited disorders : Polycystic kidney disease.
Cont.
11. Risk factor for CKD
• Vesicouterine reflux with recurrent UTI
• Obstructive uropathy
• Prior history of acute nephritis or NS
• Family H/O polycystic kidney disease.
• Renal dysplasia or hypoplasia
• Low birth weight infant
• Presence of diabetes, hypertension
• SLE, vasculitis, H/O HSP.
16. Clinical manifestations
• Chronic kidney disease (CKD) is asymptomatic in its
earliest stages (stage I and stage II), although
urinalysis findings or blood pressure may be
abnormal.
• Polydipsia and nocturia may be one of the earliest
symptoms that indicate a diagnosis of chronic kidney
disease
17. • CKD from chronic glomerulonephritis can present with
edema, hypertension, hematuria, and proteinuria.
• Congenital disorders present with failure to thrive,
polyuria, dehydration, urinary tract infection, or overt
renal insufficiency.
• Familial juvenile nephronophthisis present with
headache, fatigue, lethargy, anorexia, vomiting,
polydipsia, polyuria and growth failure over a number
of years.
Cont..
18. Systemic features
Advanced chronic kidney disease ( stage 3-5) may include the
following:
• Growth retardation
• Fatigue
• Hypertension
• Anemia
• Renal osteodystrophy
• Severe acidosis
• Hyperkalemia
• Left ventricular failure & pulmonary oedema.
32. Investigations Findings
CBC Normocytic normochromic anemia
S. creatinine Raised
S. Uric acid Raised
S. electrolyte Hyperkalemia, Hypernatremia(loss of free
water),Hyponatremia(volume overloaded),
Acidosis
S. calcium Low
S. phosphate Raised
33. Investigations Findings
S. PTH level Raised
S. 1,25-dihydroxy-vitamin D Reduced
S. Lipid profile Raised serum cholesterol,
Raised serum triglyceride
S. albumin Low (heavy proteinuria)
S. Iron studies Reduced serum iron, ferritin,
transferrin
S. C3, C4, ANA Decreased C3,C4,
ANA positive
34. Investigation Findings/Uses
Urine R/M/E Specific gravity- Low
Protein, Pus cell, RBC, RBC cast.
USG of KUB region
with MCC and PVR
Hydronephrosis, Small echogenic kidneys (in
advanced renal failure), Polycystic kidneys,
Obstruction in urinary tract.
DTPA renogram GFR measurement, Obstruction in urinary tract,
Renal blood flow assessment.
DMSA Renal scarring
36. Glomerular filtration rate
• Modified Schwartz formula
K × Height in cm
Serum creatinine in mg/dL
It’s a bedside formula that estimates GFR (15 - 75 mL/min/1.73m2)
• Endogenous creatinine clearance
Widely used but shows variable and inaccurate results.
• More accurate measures
By measuring plasma clearance of Inulin, DTPA, Iohexol,
Cystatin C
eGFR(mL/min/1.73m²)=
37. Fig: DMSA(dimercaptosuccinic acid) scan showing multiple focal cortical defects in
the upper and lower poles of the left kidney (posterior view)
Lt Rt
38. Fig: Renal imaging in posteroanterior position (R = right kidney, L = left kidney).99mTc-DTPA renal
scintigraphy shows vascular bed over the left kidney without visualization of the parenchyma with practically
afunctional Reno graphic curve of the same kidney
39. Reduced GFR
or
Proteinuria, Hematuria
Renal USG
Contracted
kidneys
Hydronephrosis/
Abnormal bladder
Normal kidneys
MCUG, DMSA
Neurogenic
bladder,
Obstructive
uropathy
Chronic
glomerulonephritis
MCUG Renal biopsy
Reflux
nephropathy
Fig: Algorithm of approach to diagnosis of chronic kidney disease
41. Fluid and electrolyte management
Fluid
• Fluid restriction isn’t necessary until development of ESRD.
Stage 1-4 : Input should match urine output
Stage 5 : Input should match (volume removed by
dialysis +any remaining Urine output).
Sodium
• Usual sodium intake is allowed with no extra salt.
• Sodium supplements : polyuria with urinary sodium loss.
• Sodium restriction & diuretic therapy: high BP,
edema or heart failure
42. contd..
Potassium
• In most children potassium balance is maintained and
restriction not needed until GFR is less than 10
ml/min/1.73m²
• In case of hyperkalemia : Restriction of dietary K+ intake.
Oral alkalinizing agent.
Potassium exchange resins.
• In case of hypokalemia : Supplements of food articles rich
in potassium (fruits, almonds,
green vegetables) are allowed.
43. Nutrition
Assessment of growth and nutritional status
• 6 monthly done usually.
• 1-3 monthly in children with polyuria, severe malnutrition,
growth failure, undergoing dialysis.
Dietary plans
• Calorie intake- Carbohydrate 55-60%+ Fat 30%+Protein 10%.
• Energy intake equivalent to Recommended dietary allowance
(RDA) of a healthy child of same age.
• If patients is incapable to maintain oral feed- NG feeding.
44. Protein
• Low protein diet is not recommended
• Diet protein content- 100% of RDA of protein
• At least 50% should be high biological value proteins
• Patients on dialysis- additional 0.4-0.8 g/kg/day intake.
Fat
• Saturated fat- comprise less than 10% of total calories
• Preferred dietary fat- polyunsaturated fats,
medium chain TGs
45. Micronutrients
• 100% RDA of Vit A,B1,B2,B6,B12,C,E,K,Folic acid,Cu,Zn
should be taken.
• Children on Peritoneal dialysis need vit C, pyridoxine & Folic
acid supplementation.
• Excess vitamin C is avoided- as it cause oxalate deposition in
kidney
46. Anemia
Correction of anemia can be done by-
• Correction of deficient factors: Iron, Folic acid etc.
• Recombinant human erythropoietin (rHuEPO) therapy
Initiated when Hb% is below 10 g/dL.
Dose :50-150 mg/kg/dose (s/c 1-3 times weekly).
Concurrent iron supplementation has to be given.
• Darbepoetin alfa
A longer-acting agent than rHuEPO
Dose: 0.45 μg/kg/wk (can be used once a month also)
Advantage: extended duration of action.
47. Transferrin <20% / Ferritin<100 ng/ml Transferrin>20% + Ferritin>100 ng/ml
No other cause
Iron deficiency
Oral iron I/V iron Anemia of chronic disease
Continue maintenance therapy
Evaluate for causes of anemia
PBF, Reticulocyte count, RBC indices, Iron studies(s. ferritin , s. transferrin)
If CKD stage 3-5
Start oral iron and folic acid
Anemia
Erythropoietin
Yes
No
Pre-dialysis Hemodialysis
Refractory Refractory
Improvement
Refractory
Fig: evaluation and management of anemia in children with CKD
48. Hypertension
Management options are as follows
• In case of suspected volume overload
Salt-restricted diet (<2 g/day)
Diuretic therapy
• In case of proteinuric renal disease
ACE inhibitors (enalapril, lisinopril)
Angiotensin II receptor blockers (losartan)
• Adjunctive agents (If blood pressure still not controlled)
CCB, β-blocker, direct vasodilators
49. Contd….
Regarding diuretics
• CKD stages 1-3: Thiazide diuretics are initial choice
• Beyond CKD stage 3: Thiazides are ineffective and loop
diuretics become drug of choice.
Regarding ACE inhibitors and ARBs
They have the potential ability to decrease proteinuria and slow
the progression to ESRD.
Cautions to be taken during using these drugs
Renal function and blood electrolyte monitoring should be done
50. Growth failure
Initial measure
• Resolving Nutritional deficiencies, acid-base imbalance, salt
depletion, calcium and vit D deficiency.
Recombinant growth hormone (rhGH) therapy
• Children who remain less than −2 SD (HFA)despite optimal
medical support.
• Initial dose (0.05 mg/kg/24 hour), subcutaneously.
51. Recombinant growth hormone (rhGH) therapy
contd..
Contraindications
• Uncontrolled diabetes
• Severe osteodystrophy
• Active malignancy
Adverse effects
• Hyperglycemia
• Avascular hip necrosis
• Hypertension
• Pseudotumor cerebri
Therapy discontinuation
• Patient reaches 50th
percentile for MPH
• Achieves a final adult height
• Undergoes renal
transplantation
• Closed epiphysis
• Hypersensitivity
52. Treatment of hyperphosphatemia
Dietary phosphate
• In CKD stage 3 to 5: 80% of RDA should be given.
• Phosphorous rich foods should be avoided (e.g. red meat,
chocolate, cola drinks, nuts, beans, peas, etc.)
• Oral phosphate binders: Used when dietary restriction fails
to maintain normal phosphate level.
54. Renal Osteodystrophy
Goals of treatment
To prevent bone deformity and to normalize growth velocity.
Target phosphorus level
For Adolescents: (3.5 - 5.5 mg/dL)
For 1-12 yrs of age: (4-6 mg/dL)
Management options
Low-phosphorus diet
Phosphate binders (enhance GI excretion)
Vitamin D administration
55. Vitamin D administration
Indications
1,25-dihydroxy-Vitamin D level below the normal range.
and/or
PTH level is above the goal range for particular stage of CKD.
Desired level of calcium-phosphorus product(Ca x PO4)
• <55 mg2 /dl2 in adolescents
• <65 mg2/dL2 in younger children
56. Management of Acidosis
• Mild acidosis may not require correction.
• If HCO3 is less than 15 mEq/L, oral supplementation is
given with either sodium citrate or sodium bicarbonate.
• The target serum HCO3 level >22 mEq/L.
Adjustment In Drug Dose
• Dosage adjustment needed as drug excretion is hampered.
• Adjustment include lengthening of the interval between
doses or decreasing the absolute dose or both
57. Management of Insulin resistance, hyper-
lipidemia and preventing CVS complications
• Target level
Total cholesterol: <170 mg/dl, LDL level: <110 mg/dl
• Lifestyle modification
Increased physical activity, intake of high amount of fiber.
• For hyperglycemia
Insulin can be used. Metformin may be used sometimes.
• Prevention of cardiovascular complications
Management of hypertension, avoiding fluid overload, regulation
of calcium-phosphate-PTH axis.
58. IMMUNIZATIONS
• Child with CKD should receive all standard immunizations
according to the schedule used for healthy children.
Withholding live virus vaccines
CKD related to glomerulonephritis,
During treatment with immunosuppressive medications.
Before kidney transplantation
Vaccines for measles, mumps, rubella, and varicella
has to be given
Influenza vaccine
Yearly administration
59. End-Stage Renal Disease
Its the state in which a patient’s renal dysfunction has progressed
to the point at which homeostasis and survival can no longer be
sustained with native kidney function and maximal medical
management.
Management
Renal replacement therapy
Dialysis (Peritoneal dialysis/Hemodialysis)
Renal transplantation
61. Contd..
Peritoneal dialysis Hemodialysis
Complications contd..
Hypotension + ++
Hypothermia - -
Central line infection - +
Inguinal or abdominal hernia + -
Peritonitis + -
Protein loss + -
Respiratory compromise + -
Vessel thrombosis - +
62. Slowing the Progression of Disease
• Optimal control of hypertension(<75th percentile)
• Reduction of proteinuria with ACE inhibitors/ ARBs
• Maintaining serum phosphorus level
• Prompt treatment of infections and episodes of dehydration
• Correction of anemia
• Control of hyperlipidemia
• Administrating vitamin D
• Preventing obesity
• Avoiding the use of NSAID and nephrotoxic drugs
63. Conclusion
Chronic kidney disease can shorten lifespan of a child but
proper management of this condition can enable a child to
lead a normal or near normal life.
World kidney day (WKD) will be observed worldwide on
14th march 2019, this year’s theme for WKD is Kidney
Health for Everyone Everywhere