clinical CASE STUDYPRESENTATION

R.VINOEDH NAIDU @ PRP U41HSB (2014/2015)PRECEPTOR : MISS JESSICA

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

DIAGNOSIS:

NEPHROTIC SYNDROME WITH SPONTANEOUS REMISSION

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

Etiology

• Genetic

• Secondary

• Idiopathic or Primary

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.

Nephrotic syndrome in childhood Response to 4 weeks of daily steroids

ISKDC

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