15-Nephrology.ppt training mannual for public health officers

Handout

1. Pediatrics Nephrology
Compiled by Dr. Begna(GP)
9/12/2021 1

2. 1.AKI
2. CKD
3.GLOMERULAR DISEASES
Nephrotic syndrome
 Nephritic syndrome
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3. AKI
•Abrupt loss of kidney function leading to a rapid
decline in the GFR, accumulation of waste products
•Affect all ages
•The incidence varies from 2–5% of all hospitalizations
to > 25% in critically ill infants and children
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4. •A classification system proposed by the Kidney
Disease Improving Global Outcomes (KDIGO) AKI
Consensus Conference takes both serum creatinine
and urine output criteria into account to define and
stage AKI
•Definition: Increase in serum creatinine by
≥
0.3 mg/dL from baseline within 48 hr; or
Increase in serum creatinine to 1.5 times
≥
baseline within the prior 7 days; or Urine
volume 0.5 mL/kg/hr for 6 hr
≤
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5. Causes of AKI conventionally classified
into 3 categories
•Pre-renal
•Intrinsic renal
•Post-renal
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6. Prerenal AKI
•Prerenal azotemia
•Diminished effective circulating arterial volume-------
leads to inadequate renal perfusion and a decreased
GFR
•Common causes----dehydration, sepsis, hemorrhage,
severe hypoalbuminemia, and cardiac failure
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7. Intrinsic renal AKI
•Disorders that causes renal parenchymal damage,
including sustained hypoperfusion and ischemia
•Common causes….GN, HUS, ATN, Cortical necrosis,
renal vein thrombosis, rhabdomyolysis, acute
interstitial nephritis, tumor infiltration, Toxin and
drugs
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8. Postrenal AKI
•Disorders characterized by obstruction of the urinary
tract----it should be bilateral
•Common causes:
neonates & infants-----posterior urethral valves &
bilateral ureteropelvic junction
obstruction(congenital)
older children & adolescents--urolithiasis, tumor,
hemorrhagic cystitis, & neurogenic bladder
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9. Dx
•Hx ----very important in dx of cause
•P/E
•Lab: CBC, U/A, serum electrolytes, RFT,
Abdominal U/S,
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10. Common Complications of AKI
•Hyperkalemia
•Pulmonary edema
•Hypertension
•Myocardial infarction
•Pulmonary embolism
•Anemia---dilutional
•Nausea—vomiting
•Seizure
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11. Treatment of AKI
•Treat the underlying cause
•Treat complications
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12. Pediatric CKD
•Criteria: NKF KDOQI Guidelines
 Kidney damage for ≥ 3 mo, as defined by
structural or functional abnormalities of the
kidney, with or without decreased GFR,
GFR < 60 mL/min/1.73 m2 for ≥ 3 mo, with or
without the other signs of kidney damage
described above
•The pediatric CKD prevalence is approximately 18 per
1 million children
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13. Stages of CKD: NKF KDOQI Guideline
Stages Description GFR (mL/min/1.73 m2)
1 Kidney damage with normal or
increased GFR
≥90
2 Kidney damage with mild decrease
in GFR
60-89
3 Moderate decrease in GFR 30-59
4 Severe decrease in GFR 15-29
5 Kidney failure <15 or on dialysis
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14. Etiology
•Congenital, acquired, inherited, or metabolic renal
disease
•Non-glomerular or glomerular in origin
•In < 5 yr of age:
most commonly---congenital abnormalities---renal
hypoplasia, dysplasia, or obstructive uropathy
often diagnosed with prenatal ultrasonography
•>5 yr of age----acquired or inherited forms of
glomerulonephritis predominate
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15. Clinical features
•Varied and depends on the underlying etiology and
CKD stage
•Growth failure, vomiting, polyuria---in some genetic
forms
•edema, hypertension, hematuria, and proteinuria---
in glomerular forms
•In advanced CKD---uremic symptoms i.e., worsening
fatigue, weakness, nausea, vomiting, anorexia, and
poor sleep patterns
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16. Ix
•Abdominal U/s
•RFT---rise in BUN and crea
•U/A---Hematuria, proteinuria
•Serum electrolyte: Hyperkalemia, hypo/hypernatremia,
hyperphosphatemia, hypocalcemia, increase in uric acid
•Serum albumin----hypoalbuminemia(in heavy proteinuria)
•Lipid profile----dyslipidemia
•GFR
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17. Imaging
•Bilaterally small kidneys with thinned cortices
suggest intrinsic disease like GN
•Unilateral small kidney may indicate renal
arterial disease
•Clubbed calyces and cortical scars suggest
chronic infection, ischemia
•Enlarged cystic kidneys suggest cystic kidney
disease
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18. GFR
•Estimate renal function
•Inulin clearance is the gold standard to measure the GFR---
but it is no longer readily available
•Other methods for measuring the GFR--- iohexol or various
radioisotopes, endogenous markers (e.g., creatinine and/or
cystatin C
•A new bedside creatinine-based estimating equation of
estimated GFR (mL/min/1.73m2 ) = 0.43 × height (cm)/serum
creatinine (mg/dL)
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19. Rx
•Supportive
•Fluid and electrolyte Mgt
•Rx of HTN, proteinuria
•Drug dose adjustment
•Dialysis
•Renal transplantation
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20. Glomerular Diseases
•May be a result of genetic, immunologic, perfusion,
or coagulation disorders
•Glomerulonephritis(GN)
immunologic injury to the glomerulus---by two major
mechanisms---deposition of circulating immune complexes
+ interaction of Ab with glomerular Ag
 generic term for several diseases--more precisely a
histopathologic term defining inflammation of the
glomerular capillaries
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21. Nephrotic syndrome(NS)
•A glomerular disease
•Characterized by nephrotic range proteinuria and the triad of
hypoalbuminemia(<2.5gm/dl), edema, & hyperlipidemia
(cholesterol > 200mg/dl).
•Incidence : 2-7 per 100,000 pediatric population
•Nephrotic range proteinuria------protein excretion of >
40
 mg/m
2
/hr or 3.5 gm/ 24 hrs or morning urine protein : 
creatinine ratio of > 2-3 : 1.
•More common in boys than in girls----2 : 1
• Most commonly appears b/n the ages of 2 and 6
 yr
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22. Etiology
•Primary/idiopathic----MC
minimal change disease (the MC), focal segmental
glomerulosclerosis, membranoproliferative GN, C3
glomerulopathy, and membranous nephropathy
•Secondary to systemic diseases like SLE, Henoch-Schönlein
purpura, malignancy (lymphoma and leukemia), and
infections (hepatitis, HIV, and malaria)
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23. Minimal change NS (MCNS)
• About 85% of total cases of NS in children
•The glomeruli appear normal or show a minimal
increase in mesangial cells and matrix
•More than 95% of children with minimal change
disease respond to corticosteroid therapy
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24. Pathophysiology
• Increased permeability of the glomerular capillary
wall-------------------leads to massive proteinuria &
hypoalbuminemia
•Idiopathic nephrotic syndrome is associated with complex
disturbances in the immune system, especially T cell–
mediated immunity
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25. Clinical feature
•Edema, which is initially noted around the eyes and in the
lower extremities
•Exact mechanism of edema formation is unknown…. two
opposing theories, the underfill hypothesis and the overfill
hypothesis
•Underfill hypothesis---loss of protein---decrease oncotic
pressure----fluid shift from intravascular to interistitial
•Overfill hypothesis---- primary sodium retention, with
subsequent volume expansion and leakage of excess fluid into
the interstitium
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26. Complications
•Hyperlipidemia
•Infections
•Hypercoagulability
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27. Hyperlipidemia
•Thought to be the result of increased synthesis as
well as decreased catabolism of lipids
•Although commonplace in adults, the use of lipid-
lowering agents in children is uncommon
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28. Increased Susceptibility to
Infections
•Cellulitis, spontaneous bacterial peritonitis, and
bacteremia
•As a result of hypoglobulinemia----urinary losses of
immunoglobulin, complement factors
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29. Hypercoagulability
•NS is a hypercoagulable state, why?
•Vascular stasis from hemoconcentration and intravascular
volume depletion
•Increased platelet number and aggregability, and changes in
coagulation factor levels
•Increase in hepatic production of fibrinogen
•Urinary losses of antithrombotic factors such as antithrombin
III and protein S
•DVT risk is low in children---2 to 5%
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30. Ix
•U/A: +3 or 4+ proteinuria
microscopic hematuria is present in 20% of children
a spot urine protein : creatinine ratio should be > 2.0.
•RFT: serum crea-normal
•Serum electrolytes
•HBsAg, HCV Ab, PICT
•Serum albumin<2.5g/dl
•Serum cholesterol and triglyceride levels are elevated
•Serum complement levels are normal
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31. Rx
•Children with their first episode of nephrotic syndrome and
mild to moderate edema may be managed as outpatients
•Immunosuppressive therapy---rule out TB first
•Children with onset of uncomplicated nephrotic syndrome
between 1 and 8 yr of age are likely to have steroid-
responsive MCNS, and steroid therapy may be initiated
without a diagnostic renal biopsy
•Children with features that make MCNS less likely (gross
hematuria, hypertension, renal insufficiency,
hypocomplementemia, or age < 1 yr or > 12 yr) should be
considered for renal biopsy before treatment.
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32. •Prednisone or prednisolone should be administered
as a single daily dose of 60 mg/m2 /day or 2
mg/kg/day to a maximum of 60 mg daily for 4-6 wk
followed by alternate-day prednisone (starting at 40
mg/m2 qod or 1.5 mg/kg qod) for a period ranging
from 8 wk to 5 mo, with tapering of the dose
•Approximately 80–90% of children respond to
steroid therapy
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33. •Response is defined as the attainment of remission
within the initial 4 wk of corticosteroid therapy
•Remission consists of a urine protein : creatinine
ratio of < 0.2 or < 1+ protein on urine dipstick testing
for 3 consecutive days.
•Most children with minimal change disease respond
to daily prednisone therapy fairly quickly, within the
first 2-3 wk of treatment.
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34. •Relapse is an increase in the first morning
urine protein : creatinine ratio > 0.2 or a
reading of 2+ and higher for 3 consecutive
days
•Frequently relapsing is two or more relapses
within 6 mo after the initial therapy or four
relapses in a 12-mo period
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35. •Steroid dependent is a relapse during steroid
tapering or a relapse within 2 wk of the
discontinuation of therapy
•Steroid resistance is the inability to induce
remission within 4 wk of daily steroid therapy
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36. Mgt of edema
•Severe symptomatic edema: plural effusion,
ascites, genital edema
•Admit to hospital
•Sodium, water/fluid restriction
•Elevate scrotum
•+ loop diuretics (furosemide): oral , IV;
caution should be exercised!
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37. Nephritic syndrome
•It is a syndrome characterized by proteinuria,
hypertension, oliguria, hematuria, RBC casts and
edema
•Progressive decline in GFR also occurs
•Several conditions can lead to this clinical syndrome
•The prototype condition for nephritic syndrome is
post streptococcal glomerulonephritis
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38. Acute Poststreptococcal
Glomerulonephritis-APSGN
•A classic example of the acute nephritic syndrome
•Follows infection of the throat(1-2wk) or skin(3-6wk)
by certain nephritogenic strains of group A β-
hemolytic streptococci----M strains
•Characterized by the sudden onset of gross
hematuria, edema, HTN, and renal dysfunction
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39. •Epidemics and clusters of household
•97% of cases occur in less developed
countries.
•More common in children 5-12 yr old
•Uncommon before the age of 3 yr
•More common in males
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40. Clinical manifestations and Hx
•Pt develops an acute nephritic syndrome 1-2 wk after
an antecedent streptococcal pharyngitis or 3-6 wk
after a streptococcal pyoderma
•The history of a specific infection may be absent
•Asymptomatic microscopic hematuria with normal
renal function to gross hematuria with acute renal
failure
•Edema, hypertension, and oliguria
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41. •Peripheral edema typically results from salt and
water retention and is common;
•Nephrotic syndrome develops in a minority (<5%) of
childhood case
•Acute phase generally resolves within 6-8 wk.
•Urinary protein excretion and HTN usually normalize
by 4-6 wk after onset
•Persistent microscopic hematuria can persist for 1-2
yr after the initial presentation
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42. Ix
•U/A= RBCs, RBC casts, proteinuria, and polymorphonuclear
leukocytes
•CBC= a mild normochromic anemia may be present from
hemodilution and low-grade hemolysis
•C3, CH50 decreased
•C4 normal
•Confirmation of the dx requires clear evidence of a prior
streptococcal infection
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43. ddx
•Systemic lupus erythematosus
•Endocarditis
•Membranoproliferative GN
•Acute exacerbation of chronic GN
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44. Complications
•Encephalopathy(10%) and/or heart failure secondary
to hypertension or hypervolemia
•Hypertensive encephalopathy must be considered in
pts with blurred vision, severe headaches, altered
mental status, or new seizures
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45. Mgt
•Supportive---no specific therapy for PSGN
• Mgt of HTN and edema
•Sodium and water restriction and loop diuretics
• Loop diuretics generally provide a prompt diuresis
with reduction of blood pressure and edema
•IV furosemide is given at an initial dose of 1 mg/kg
(maximum 40 mg)
•Pts with hypertensive encephalopathy may require
additional antihypertensive agents---oral nifedipine
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46. •A 10-day course of systemic antibiotic therapy
with penicillin is recommended to limit the
spread of the nephritogenic organisms, but
antibiotic therapy does not affect the natural
history of APSGN
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47. Prognosis
•Complete recovery occurs in > 95% of children
with APSGN
•Recurrences are extremely rare.
•Mortality in the acute stage can be avoided by
appropriate management of acute renal failure,
cardiac failure, and hypertension.
•Infrequently, the acute phase is severe and leads
to glomerulosclerosis and chronic renal disease in
< 2% of affected children.
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48. References
•Nelson Textbook of Pediatrics, 21th ed
•Up-to-date 2018
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