Vinoedh Naidu @ nephrotic syndrome
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Transcript of Vinoedh Naidu @ nephrotic syndrome
OBJECTIVES :
1. Review
–Epidemiology & histopathology
–Signs and symptoms
–Treatments
2. Discuss
* Pharmacotherapy
SOCIAL HISTORY:
No family history of known medical illnessEldest son and has 3 y.o brother
REVIEW OF SYSTEM:
BP : 114 /73 mmHgPR : 80 beats/minT : 37 C̊O / E : Alert , conscious
PATIENT DEMOGRAPHIC:
Name : M.I.NAge : 5 y.oGender : MaleRace : MalayDOA : 25 /03/ 2015DOD : 03 /04/ 2015
ALLERGY :
NKMI / NKDA
HISTORY OF PRESENTING ILLNESS:
1st incident of periorbital puffiness 3/12,Coughing and running nose 1/52Bilateral periorbital swelling and facial puffiness 3/7Usually happen after waking up in the morning and resolves in the evening. Father also noted child had bilateral pedal edema on admission day. Looks chubby than usual since day ago
CHIEF COMPLAINT :
Bilateral periorbital swellingFacial puffiness & mild pedal edema
Referred from private GP
Definition
• Manifestation of glomerular disease, characterized by nephrotic range proteinuria associated with large urinary losses of protein : hypoalbuminaemia , edema and hyperlipidemia
- Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1801
EPIDEMIOLOGY
• 2 – 7 cases per 100,000 children per year• Higher in underdeveloped countries• Occurs at all ages but is most prevalent in children
between the ages 1-6 years.• It affects more boys than girls, 2:1 ratio
http://www.kidney.org/site/107/pdf/NephroticSyndrome.pdf
Complex disturbances in immune system
Genetic Mutations / Mutations in proteins
Increased permeability of the glomerular capillary wall
Massive proteinuria
Hypoalbuminaemia
Edema
DIAGNOSIS / SIGNS & SYMPTOMS
• Edema (gut, Facial, pedal)• Proteinuria• Abdominal discomfort due to oedema• Bacterial peritonitis (pulmonary, cardiac)• Poor appetite
VITAL SIGNSNORMAL RANGE
17/3 18/3 19/3 20/3 21/3 22/3 25/3 31/3 1/4 2/4 3/4
BP <110/70 113/73
97/67 107/60 110/73106/68
110/66109/62
93/6396/62
106/72 99/60100/70
112/7597/58
118/70109/61
103/6295/65
TEMP AFEBRILE
RR 20-25 26
PR 76-106 104 94 75
REFERENCE RANGE CLINICAL VALUE
18/3/15 22/3/15 26/3/15
Hb 11.5-16.5g/100ml 14.9 14.4WBC 4-11x 10/L 11.1 11.2Platelet 150-410 X 10/L 409 401T.Protein 66-87 g/L 61Albumin 35-50 g/L 17 20 25T. Bilirubin <20 umol/L 3ALP 53-141 u/L 209ALT <32u/L 15Creatinine 64-122 umol/L 19 16 19Blood Urea 1.7-8.3 mmol/L 4.6 4.0 5.1Na 135-145 mmol/L 136 137 135K 3.5-5.0 mmol/L 4.2 4.0 3.8Cl 96-106 mmol/L 109 107 104Cholestrol 11 12.4Triglycerides 3.2 3.5Urine protein 0.05 – 0.08 1.4 0.54ASOT -VEC3 0.9-1.8 1.3C4 0.1-0.4 0.27
PCI : 0.89
Lab Investigations• Urine Examination• Complete Blood Count & Blood picture• Renal parameters :
– Urine Protein : Creatinine ratio / 24h urine protein– Urea & electrolytes
• Liver Function Test– Albumin
URINE DIPSTICK18/3 19/3 20/3 21/3 22/3 23/3 27/3 28/3 1/4 2/4
3+ 2+ 2+ 1+ NIL 2+ 2+ - -
“When bubbles settle on the surface of the urine, it indicates disease of the kidneys and that the complaint will be protracted”
Hippocrates
Additional TestsAdditional Tests• Antinuclear factor / anti-dsDNA*• C3 and C4 levels *• Antistreptolysin O (ASOT) *
Ghai Essential Paediatrics,8th edition, page 478
Indications for BiopsyIndications for Biopsy
• Age below 12 months• Gross or persistent microscopic hematuria• Hypertension• Impaired renal Function• Failure of steroid therapy *
Nutritional deficiencies - Kwashiorkor, brittle hair and nails, alopecia, stunted growth, demineralization of bone
Spontaneous peritonitis may occur and opportunistic infections are prevalent.
Hypertension with cardiac and cerebral complications in patients with diabetes or collagen vascular disease.
Hypovolemia - oliguria, abd pain, anorexia, postural hypotension
COMPLICATIONS
30
MEDICATION CHARTDRUGS 18/3 19/3 20/3 21/3 22/3 23/3 25/3 26/3 27/3 28/3 29/3 30/3 31/3 1/4 2/4 3/4
IV C-Penicillin 960,000 u QID
Sy. Penicillin V125mg BD
IV Frusemide 20mg STAT
IV Frusemide 20mg BD
T. Prednisolone 25mg OM, 20mg ON
C-Penicillin - 30mg/kg QIDPenicillin V – 125mg BD (1-5 years)
250mg BD (6-12 years) 500mg BD (>12 years)
IV Frusemide – 1mg/kg/doseT. Prednisolone – 60mg/m2/day
Initial Episode• High protein diet• Salt moderation• Treatment of infections• If significant edema – diuretics• Corticosteroid therapy* with Prednisolone
– 60mg/m2/day* for 4weeks (-> fail : STEROID RESISTANT NS*)– 40mg/m2/EOD for 4weeks– ↓ 25% dose monthly over next 4 months
PAEDIATRIC PROTOCOL, MOH
80% REMISSION !!! *
Subsequent course• Relapse
– Infrequent Relapsers : 3 or less relapses per year– Frequent Relapsers : 4 or more relapses per year* (0.1-0.5mg/kg/dose
for 6 months)
• Steroid therapy– Steroid dependant : relapse following dose reduction or discontinuation– Steroid resistant : Partial or no response to initial treatment
• Steroid toxicity :» Cyclophosphamide (2-3mg/kg/day 8-12weeks) *»
ROLE OF PHARMACISTCounseling on Steroids :
1) Indications, dose, frequency & duration
2) Side effects of steroids
3) Importance of compliance
4) Need of coming to hospital when relapse / infection
5) Ensure proper understanding
Side Effects With Long Term Use of Steroids “Steroid toxicity
1) Hyperglycemia & ↑ appetite (↑ weight)
2) Cushing Syndrome
3) ↑ GI symptoms
4) Osteoporosis
5) ↓ skin thickness (dermatitis)
6) Cataract & glaucoma
7) ↓ immunity (infection risk)
8) Gross / scrotal edema
HOME MONITORINGHome monitoring of urine protein and fluid status is important.
Parents should be trained to monitor first morning urine by dipstick.
Record of daily weight,urine protein and steroid dose should be kept in log book.
Any increase in urine protein or daily weight should be reported as early as possible.
Cochrane meta-analysis: steroid• In children in their first episode, treatment with prednisone for at least three
months results in fewer children relapsing by 12 to 24 months with an increase in benefit being demonstrated for up to seven months of treatment compared with two months therapy. In a population with a baseline risk for relapse of 60% with two months of prednisone, daily prednisone for four weeks followed by alternate-day therapy for six months would be expected to reduce the number of children experiencing a relapse by about 33%.
• In comparison with 3 months of therapy, six months of therapy results in a reduced risk for relapse without increase in adverse effects.
• The reduction in risk for relapse is associated with both an increase in duration and an increase in dose.
• During daily therapy, prednisone is as effective when administered as a single daily dose compared with divided doses.
• Alternate-day therapy is more effective than intermittent therapy (3 consecutive days of 7 days) in maintaining remission.
REFERENCES• Paediatric protocol, MOH (2012)
• Lau, Keith, et al. "Steroid Responsive Nephrotic Syndrome in IgA Nephropathy with FSGS." The
• Internet Journal of Nephrology 4 (2008): n. pag. Print.
• "Pediatric Nephrotic Syndrome." Pediatric Nephrotic Syndrome. N.p., n.d. Web. <http://
• emedicine.medscape.com>.
• USA. NIH. NIDDK. Childhood Nephrotic Syndrome. N.p.: n.p., 2008. Print.
• Trachtman, Howard. “Common Diseases: Minimal Change Nephrotic Syndrome.” Nephrology
• Self Assessment Program 11 (2012) 19-20. Print.
• Trachtman, Howard. “Common Diseases: Focal Segmental Glomerulosclerosis.” Nephrology