NEPHROTIC SYNDROME
THE SWOLLEN CHILD
DEFINITION
It is a clinical syndrome of:
1.	Heavy proteinuria
>1 g/m2/day
2.	Hypoproteinemia
↓ serum albumin < 2.5 g...
EPIDEMIOLOGY
West
 Uncommon: 3 new cases per 100,000 child population
Asian
Higher incidence: 16 new cases per 100,000 chi...
CLASSIFICATION
Minimal Change Disease
 most common (70-80%)
 M:F = 2:1
 < 7 years old
steroid-sensitive nephrotic syndrome
do not prog...
PATHOPHYSIOLOGY
Primary disorder:
Edema:
- under fill theory: hypoalbuminemia
- over fill theory:↑ tubular NaClreabsorption secondary to RAAS -> intravas...
COMPLICATION
Infection
Spontaneous bacterial peritonitis, cellulitis, bacteriemia (S.pneumoniae, E.coli)
Steroid and immun...
CLINICAL MANIFESTATION
Sudden onset of  dependent pitting oedema
   - periorbital
   - scrotal or vulva
   - ankle or leg
...
HISTORY TAKING
First time or relapse???
History of edema noted on awakening in the morning  or sudden swelling??
Distribut...
PHYSICAL EXAMINATION
Assessment of hydration status identifies fluid imbalances (dehydration, overhydration)
Blood pressur...
DIFFERENTIAL DIAGNOSIS
Main ddx:
Anaphylaxis
Cellulitis (orbital,periorbital)
Angioedema
Nephrotic synd.
Other causes of h...
INVESTIGATIONS
Diagnostic studies:
Proteinuria +1> on 2/3 random urine sample (Dipstick)
P:C (> 200mg/mmol) (early m...
Other investigations
 complement levels: decrease suggest other thn MCD
Antistreptolysin O titre and throat swab
Hepatitis...
DEFINITION FOR DX & TX OF IDIOPATHIC NS
 
REMISSION:
Urinary protein excretion < 4 mg/m2/hour or urine dipstix  nil...
MANAGEMENT
STEROID SENSITIVE
Prednisolone regime for initial dx:
60 mg/m2/day (max 80mg/day) for 4 weeks
40 mg/m2/48 hr (m...
SCHEMA OF TREATMENT OF IDIOPATHIC NEPHROTIC SYNDROME
 
1. Nephrotic Syndrome
   	    Initial Diagnosis
    Prednisolone 60...
STEROID RESISTANT
Symptomatic therapy:
diuretic
blood pressure control : ACEi (captopril, enalapril), angiotensin II  	rec...
Steroid toxicity
Stunting of growth 
Cataracts
Striae
Severe cushingoid features
behavioural changes, a rounded face, cent...
MANAGEMENT
Mx of oedematous state
Bed rest to be avoided as there is a tendency of hrpercoagulability
Dietary advice: no a...
Mx complication:
Infection: parenteral penicillin and a third generation cephalosporin (in primary peritonitis)
If exposed...
URINE ALBUMIN  MONITORING
 
It is advocated that monitoring of urine albumin excretion be done regularly either at home  w...
EDUCATION
Education:
Parents and school teachers  should be provided with information regarding the disease which includes...
PROGNOSIS
Acute nephritic syndrome
NUR AMIRA BINTI MOHD ASRI
CASE SCENARIO
    A 7 year old Malay boy was admitted 3 days ago with the chief complaints of facial puffiness and passing...
NEPHRITIC SYNDROME
It is a clinical complex, usually acute onset, characterized by:
oedemaeg facial puffiness
Microscopic/...
The lesions that cause nephritic syndrome have in common proliferation of cells within in glomeruli, accompanied by a leuk...
NEPHROTIC SYNDROME
GLOMERULONEPHRITIS
Acute poststreptococcal GN
lgA nephropathy
NEPHRITIC SYNDROME
Henoch-schönleinpurpur...
POSTSTREPTOCOCCAL AGN
The commonest cause of nephritic syndrome
Usually followed a nephritogenic streptococcal pharyngitis...
Epidemiology of post acute strep GN
121 of the 124 nephritis patients had poststreptococcal infection. 
(Department of pae...
pathogenesis
Streptococcal infection
Immune complex formation +deposited in GBM
Complement system activated
Low serum complement
Immune...
HENOCH-SCHöNLEIN  PURPURA
It is a systemic syndrome involving the skin (purpuric rash), gastrointestinal tract (abdominal ...
Systemic lupus erythematosus (SLE)
Is an autoimmune disease that presents mainly in adolescent girls and young women(5% in...
Criteria for diagnosis of SLE
signs
Malar rash (butterfly rash)
Photosensitivity
Oral and nasopharyngeal ulcers
Pleuritis ...
lgA NEPHROPATHY
Affects children and young adults
Begins as an episode of gross hematuria that occurs within 1 or 2 days o...
Familial nephritis
The commonest familial nephritis is Alport’s syndrome
X-linked recessive disorder
Is associated with ne...
Basic workup of a child with hematuria
History-age,gender,sosioeconomicstatus,familyhistory,drug history
Physical examinat...
Dalliana Adia Bte Abd Latif
2008402292
History
Antedencenthx of streptococcal throat of skin infection- post-streptococcal GN
Ask about symptoms of swelling-faci...
Epistaxis, headache, encephalopathy- severe hypertension
Oligouria
Nonspecific symptoms eg malaise, fever, anorexia, weakn...
Investigation
Laboratory
FBC- anemia, leucocytosis
Urinalysis and culture- hematuria, proteinuria, RBCs cast,other cellula...
Acute poststreptococcal GGN- low C3, positive ASOT and anti DNAase B
TIN- hematuria, eosinophilia, sterile pyuria, low gra...
Imaging
Renal ultrasonography- usually to exclude other causes of hypertension and hematuria but usually not conducted in ...
Histology
light microscopy: lymphocytes, PMN leukocytes
Immunofluoroscene- IgG, IgA, IgM, or complement
Electron- deposit ...
Post-streptococcal nephritis
Urinalysis 
ASOT
Low complement C3 levels- return to normal after 3-4 weeks
Management
Treat the primary pathology- immunosuppressive med eg steroids or cyclophosphamide in lupus
Supportive care- Fl...
Complication
Complication of severe hypertension (e.g. cerebral haemorrhage, seizure, enchepalopathy,stroke, end organ dam...
Nephrotic and nephritic
Have a good fortune ahead!!! 
8. Nephrotic Syndrome & AcuteGlomerularNephritis
8. Nephrotic Syndrome & AcuteGlomerularNephritis
8. Nephrotic Syndrome & AcuteGlomerularNephritis
8. Nephrotic Syndrome & AcuteGlomerularNephritis
8. Nephrotic Syndrome & AcuteGlomerularNephritis
8. Nephrotic Syndrome & AcuteGlomerularNephritis
8. Nephrotic Syndrome & AcuteGlomerularNephritis
8. Nephrotic Syndrome & AcuteGlomerularNephritis
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8. Nephrotic Syndrome & AcuteGlomerularNephritis

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8. Nephrotic Syndrome & AcuteGlomerularNephritis

  1. 1. NEPHROTIC SYNDROME THE SWOLLEN CHILD
  2. 2. DEFINITION It is a clinical syndrome of: 1. Heavy proteinuria >1 g/m2/day 2. Hypoproteinemia ↓ serum albumin < 2.5 g/dL Protein:Creatinine > 200mg/mmol 3. Oedema 4. Hypercholestrolnemia > 250 mg/dL
  3. 3. EPIDEMIOLOGY West Uncommon: 3 new cases per 100,000 child population Asian Higher incidence: 16 new cases per 100,000 child population Malaysia No available data, it is thought to have higher incidence than in the west
  4. 4. CLASSIFICATION
  5. 5. Minimal Change Disease most common (70-80%) M:F = 2:1 < 7 years old steroid-sensitive nephrotic syndrome do not progress to renal failure often precipitated by respiratory infections Features: age between 1 and 10 years no macroscopic haematuria normal blood pressure normal complement levels normal renal function.
  6. 6. PATHOPHYSIOLOGY Primary disorder:
  7. 7. Edema: - under fill theory: hypoalbuminemia - over fill theory:↑ tubular NaClreabsorption secondary to RAAS -> intravascular expansion -> fluid shift following pressure gradient Hypercholesterolemia - hypopratenemia -> hepatic lipoprotein synthesis -> ↑serum lipid (cholesterol, lipoprotein) -> lipid metabolism
  8. 8. COMPLICATION Infection Spontaneous bacterial peritonitis, cellulitis, bacteriemia (S.pneumoniae, E.coli) Steroid and immunosuppressant toxicity Hypovolaemia abdominal pain and may feel faint, cold peripheries, poor pulse volume, hypotension, and haemoconcentration. A low urinary sodium (<20mmol/L) and a high packed cell volume Thromboembolism hypercoagulable state due to urinary losses of antithrombin, thrombocytosis exacerbated by steroid therapy increased synthesis of clotting factors increased blood viscosity from the raised haematocrit, This is usually arterial and may affect the brain, limbs and splanchnic circulation Hypercholesteroleamia Acute renal failure (rare)
  9. 9. CLINICAL MANIFESTATION Sudden onset of dependent pitting oedema - periorbital - scrotal or vulva - ankle or leg Weight gain Ascites - abdominal pain - malaise Diarrhea (dt intestinal oedema) Respiratory distress (dtpulm. oedema)
  10. 10. HISTORY TAKING First time or relapse??? History of edema noted on awakening in the morning or sudden swelling?? Distribution Colour changes Initiating factor? (bee sting) Painful?? Weight gain (edema) Respiratory distress Breathlessness Diarrhea Urine: frothy Pass medical and drug history: recent illness, allergies, asthma Family history
  11. 11. PHYSICAL EXAMINATION Assessment of hydration status identifies fluid imbalances (dehydration, overhydration) Blood pressure: hypertension Henoch-Schönleinpurpura (purpura) Systemic lupus erythematosus (malar rash) Rales heard on lung auscultation suggest extravascular fluid from overload or hypoalbuminemia Palpation and percussion of the abdomen may reveal ascites or masses Liver enlargement is present in several multisystem diseases (systemic lupus erythematosus, infections, polycystic disease) and in glomerulosclerosis  
  12. 12. DIFFERENTIAL DIAGNOSIS Main ddx: Anaphylaxis Cellulitis (orbital,periorbital) Angioedema Nephrotic synd. Other causes of hypoalbuminaemia Transient proteinuria Postural orthostatic proteinuria Glomerular abnormalities
  13. 13. INVESTIGATIONS Diagnostic studies: Proteinuria +1> on 2/3 random urine sample (Dipstick) P:C (> 200mg/mmol) (early morning) Serum lipid C3 level ( sensitive n specific if other than MCD) Full blood count: HCT, WBC Renal profile: normal in MCD Serum albumin: <25g/dL Urinalysis and quantification for urinary protein excretion abundant hyaline cast Haematuria (other thn MCD) Na+ <10mmol/L in hypovolaemia
  14. 14. Other investigations complement levels: decrease suggest other thn MCD Antistreptolysin O titre and throat swab Hepatitis B antigen
  15. 15. DEFINITION FOR DX & TX OF IDIOPATHIC NS   REMISSION: Urinary protein excretion < 4 mg/m2/hour or urine dipstix nil/trace for 3 consecutive days.   RELAPSE: Urinary protein excretion > 40 mg/m2/hour or urine dipstix ++ or more for 3 consecutive days.   FREQUENT RELAPSES: Two or more relapses within 6 months of initial response or four or more relapses within any 12 month period.    STEROID DEPENDENCE: Two consecutive relapses occurring during the period of steroid taper or within 14 days of its cessation. STEROID SENTITIVE: Normalization of proteinuria within 4 weeks after start of standard initial therapy with daily oral predinisolone   STEROID RESISTANCE: Failure to achieve remission in spite of 4 weeks of standard prednisolone therapy.
  16. 16. MANAGEMENT STEROID SENSITIVE Prednisolone regime for initial dx: 60 mg/m2/day (max 80mg/day) for 4 weeks 40 mg/m2/48 hr (max 60mg/dose) for further 4 weeks Prednisolone regime for relapses: 60 mg/m2/day (max 80mg/day) until remission 40 mg/m2/48 hr for 4 weeks Frequent relapse or steroid dependent: Long term low dose prednisolone for 3-6 months
  17. 17. SCHEMA OF TREATMENT OF IDIOPATHIC NEPHROTIC SYNDROME   1. Nephrotic Syndrome Initial Diagnosis Prednisolone 60 mg/m2/day (max 80/day) for 4 week   Response No Response Prednisolone 40 mg/m2/48 hours for 4 weeks   Renal Biopsy   *Discontinue *Steroid taper at 25% monthly over 4 months  2. Relapse Prednisolone 60 mg/m2/day (max 80 mg/day) till remission, then 40 mg/m2/48 hours for 4 weeks and discontinue.  3. Frequent Relapses Reinduce as for (2) above, then taper and keep low dose alternate day prednisolone at 0.1 - 0.5 mg/kg/dose for 6 months.  4. Relapse on prednisolone As for (3) if not steroid toxic, consider cyclophosphamide (cumulative dose 168 mg/kg) if steroid toxic.  5. Relapses post cyclophosphamide As for (2) and (3) if not steroid toxic. If steroid toxic, refer paediatricnephrologist to consider a). second course cyclophosphamide or b). cyclosporine therapy.
  18. 18. STEROID RESISTANT Symptomatic therapy: diuretic blood pressure control : ACEi (captopril, enalapril), angiotensin II receptor antagonist hyperlipidaemia Immunosuppressive therapy: Steroids cyclophosphamide Cyclosporin, tacrolimus, mycophenolatemofetil Indications for renal biopsy A renal biopsy is also NOT required prior to cytotoxictherapy Steroid resistant nephroticsyndrome Secondary NS Congenital NS
  19. 19. Steroid toxicity Stunting of growth Cataracts Striae Severe cushingoid features behavioural changes, a rounded face, central obesity and the tendency to bruise more easily, hirsutism Osteoporosis Proximal myopathy Recurrent infection dt low immunity
  20. 20. MANAGEMENT Mx of oedematous state Bed rest to be avoided as there is a tendency of hrpercoagulability Dietary advice: no added salt, normal protein with adequate calories Prophylactic antibiotics: oral penicillin particularly in during relapse with gross oedema Hypovolaemia: infuse salt poor albumin or 5% albumin, plasma protein derivatives or human plasma Diuretics
  21. 21. Mx complication: Infection: parenteral penicillin and a third generation cephalosporin (in primary peritonitis) If exposed to chickenpox and measles varicella-zoster immunoglobulin (VZIG) should be given within 72 hours after exposure to chickenpox / single dose of intravenous immunoglobulin. Thrombosis : Warfarin, low-dose aspirin, and dipyridamole all have been used to minimize the risk of clots.
  22. 22. URINE ALBUMIN MONITORING   It is advocated that monitoring of urine albumin excretion be done regularly either at home with urinary dipstix or at the nearest health centre.
  23. 23. EDUCATION Education: Parents and school teachers should be provided with information regarding the disease which includes: Advice and precaution of infection Danger of sudden steroid withdrawal (adrenal crisis) Immunisation: While the child is on corticosteroid treatment and within 6 weeks after its cessation, only killed vaccines may be safely be administered to the child. Live vaccines can be administered 6 weeks after cessation of corticosteroid therapy
  24. 24. PROGNOSIS
  25. 25. Acute nephritic syndrome NUR AMIRA BINTI MOHD ASRI
  26. 26. CASE SCENARIO A 7 year old Malay boy was admitted 3 days ago with the chief complaints of facial puffiness and passing smokey and frothy urine for 1 week. The facial puffiness initially started off as periorbitaloedema which then progressed to involve the entire face within a week. Urinary output was also decreased. He also complaint of fever for one week which was of low grade, intermittent with no chills and rigor. There is also presence of an erythematous itchy skin lesion on his right elbow which was first noticed 2 weeks back. There is no history of sore throat, flu, blood transfusion, nausea, and vomiting, rashes, dyspnoea and chest pain. General examinations revealed pallor, high blood pressure of 139/96 mmHg, and an erythmatous scaly circular skin lesion on his right elbow. Urine biochemistry revealed protein 3+, RBC 4+. Blood urea was raised to 500 umol/L.
  27. 27. NEPHRITIC SYNDROME It is a clinical complex, usually acute onset, characterized by: oedemaeg facial puffiness Microscopic/macroscopic haematuria (tea-coloured urine) hypertension Oligouria (decreased urine output) Azotemia/uremia (excess urea in urine)
  28. 28. The lesions that cause nephritic syndrome have in common proliferation of cells within in glomeruli, accompanied by a leukocytic infiltration. This inflammatory reaction injures the capillary walls, permitting escape of RBC into the urine(hematuria) , and induced hemodynamics changes that lead to a reduction in GFR which are manifested clinically by oligouria, reciprocal fluid retention and azotemia Hypertension is the result of the fluid retention by kidney secretion of renin.
  29. 29. NEPHROTIC SYNDROME GLOMERULONEPHRITIS Acute poststreptococcal GN lgA nephropathy NEPHRITIC SYNDROME Henoch-schönleinpurpura SLE
  30. 30. POSTSTREPTOCOCCAL AGN The commonest cause of nephritic syndrome Usually followed a nephritogenic streptococcal pharyngitis or impetigo with a strain of group A beta-hemolytic streptococci Immune-mediated inflammation Occurs most frequently in children 2 to 12 years old Boys are frequently affected This is diagnosed by evidence of a recent streptococcal infection (culture of the organism, raised ASOT) and low complement C3 levels, that return to normal after 6-8 weeks Long term prognosis is good
  31. 31. Epidemiology of post acute strep GN 121 of the 124 nephritis patients had poststreptococcal infection. (Department of paediatrics, HUSM, July 1987-June 1988) Globally-incidence has decreased in the past 3 decades Most commonly-sporadic Despite that,epidemic cases in some poor and rural communities
  32. 32. pathogenesis
  33. 33. Streptococcal infection Immune complex formation +deposited in GBM Complement system activated Low serum complement Immune injuries Cellular proliferation GBM fracture Capillary lumen narrowed hematuria proteinuria Glomerular blood flow decreased oligouria GFR low Distal sodium reabsorption Retention of water and sodium Edema and hypertension Blood volume increased
  34. 34. HENOCH-SCHöNLEIN PURPURA It is a systemic syndrome involving the skin (purpuric rash), gastrointestinal tract (abdominal pain, joints (athritis), and kidney Usually occurs between the ages of 3-10 years olds Twice as common in boys peaks during winter months Is often preceded by an upper respiratory infection Unknown cause however it is postulated that genetic predisposition and antigen exposure increase circulating lgA levels. By immunofluorescence and electron microscopy the findings may be similar to those of IgAN The combination of Skin rashes(symmetrical distributed over the buttocks,extensor surface of arms and legs and the ankles. Athralgia (knees and ankles) Periarticularoedema Colicky abdominal pain, GI petechiae,hematemesis, melaena, intussusception Glomerulonephritis
  35. 35. Systemic lupus erythematosus (SLE) Is an autoimmune disease that presents mainly in adolescent girls and young women(5% in childhood girl, rare in children younger than 9 yo, equal gender distribution in children) Multisystem disorder of unknown etiology characterized by the production of large amounts of circulating antibodies due to loss of T lymphocytes control on B lymphocytes which leads to autoantibody production Presence of multiple antibodies including antibodies to double-stranded DNA .
  36. 36. Criteria for diagnosis of SLE signs Malar rash (butterfly rash) Photosensitivity Oral and nasopharyngeal ulcers Pleuritis and pericarditis Non erosive arthritis ( more than 2 joints with effusion and tenderness. Investigation data Protenuria(>500mg/24 hrs) or RBC cellular cast in urine Positive anti-dsDNA Evidence of presence of antiphospholipid antibodies
  37. 37. lgA NEPHROPATHY Affects children and young adults Begins as an episode of gross hematuria that occurs within 1 or 2 days of a nonspecific upper respiratory tract infection. Is one the most common causes of recurrent microscopic or gross hematuria and is the most common glomerular disease revealed by renal biopsy The pathology hallmark is the deposition of lgA in the mesangium Prognosis is good in children immunofluorescence with anti-IgA antibodies deposited in the mesangium
  38. 38. Familial nephritis The commonest familial nephritis is Alport’s syndrome X-linked recessive disorder Is associated with nerve deafness and ocular defect The mother may have hematuria Can progress to end-stage renal failure by early adult life in males.
  39. 39. Basic workup of a child with hematuria History-age,gender,sosioeconomicstatus,familyhistory,drug history Physical examination-height,weight,bloodpressure,funduscopy,presence or absence of abdominal mass,skinappearance,genitalia,edema,complete physical examination. Laboratory-urinalysis (includingmicroscopic examination and RBC morphology), urine culture, complete blood count (including platelets), serum electrolytes, creatinine,calcium, serum complement, random urine for total protein, creatinine, renal imaging studies. Nelson p758
  40. 40. Dalliana Adia Bte Abd Latif 2008402292
  41. 41. History Antedencenthx of streptococcal throat of skin infection- post-streptococcal GN Ask about symptoms of swelling-facial, perioral,pedal edema, or ascites Symptom of pulmonary edema/ CHF (egdyspnoea with exertion, orthopnoea, SOB) Gross hematuria (eg dark, rust, coke, tea coloured) Family hx, other family member with nephritis or renal failure- Alport syndrome The general terms GN and nephritis are not specific enough to be very useful for treatment or prognosis!!!
  42. 42. Epistaxis, headache, encephalopathy- severe hypertension Oligouria Nonspecific symptoms eg malaise, fever, anorexia, weakness For tubulointestinal nephritis- try to obtain a history of a known etiology (eg bacterial, viral, drug related, metabolic, other) TIN, usually hx of polyuria than oliguria
  43. 43. Investigation Laboratory FBC- anemia, leucocytosis Urinalysis and culture- hematuria, proteinuria, RBCs cast,other cellular masts, pyuria? Bacteriological an serology- Anti streptolysin-O titer (>200IU/mL), Anti-DNAse B, throat swab/skin swab, lupus serology, serum IgA Measure complement level- C3 RFT- blood urea, serum creatinine, electrolytes, BUN
  44. 44. Acute poststreptococcal GGN- low C3, positive ASOT and anti DNAase B TIN- hematuria, eosinophilia, sterile pyuria, low grade proteinuria, eosinophiluria, urinary WBc casts
  45. 45. Imaging Renal ultrasonography- usually to exclude other causes of hypertension and hematuria but usually not conducted in real cut nephritic sydrome
  46. 46. Histology light microscopy: lymphocytes, PMN leukocytes Immunofluoroscene- IgG, IgA, IgM, or complement Electron- deposit in mesangial, subendothelial, or subepithelial
  47. 47. Post-streptococcal nephritis Urinalysis ASOT Low complement C3 levels- return to normal after 3-4 weeks
  48. 48. Management Treat the primary pathology- immunosuppressive med eg steroids or cyclophosphamide in lupus Supportive care- Fluid and electrolyte balance, diuretics, Ca channel blocker, ACEi, monitor rapid deterioration in renal function Diet- fluid restriction, sodium, potassium restriction, Ca supplement Dialysis
  49. 49. Complication Complication of severe hypertension (e.g. cerebral haemorrhage, seizure, enchepalopathy,stroke, end organ damage) Complication of renal failure (e.g. hyperkalemia, fluid overload, electrolyte abnormality, uremic symtoms, anemia, abnormal bone mineralization, sexual dysfunction, poor growth, anorexia) Complication of primary disease (eg SLE)
  50. 50. Nephrotic and nephritic
  51. 51. Have a good fortune ahead!!! 
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