Group Number: 8

Date: July 30, 2020

UNIVERSITY OF NORTHERN PHILIPPINES

College of Medicine
Vigan City, Ilocos Sur

A Requirement

Presented to

ILOCOS TRAINING AND REGIONAL MEDICAL CENTER

DEPARTMENT OF PEDIATRICS

By

GROUP No. 8

PATEL, ZONALI
PRAJAPATI, JAIMIN SURESHBAI
SARMIENTO, JANNINE CHRISTINE GRACE E.
SAVELLANO, PRINCESS NOREEN R.
SOLANKI, JAY JITENDRABHA

A.Y 2020 – 2021

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Date: July 30, 2020

General Data: 7, F
Salient features:
HPI:
• Facial edema & pitting bipedal edema
PE:
• BP: 140/90 – Hypertensive (Normal BP for age, SBP: 100-120; DBP: 60-75)
• RR: 30 breaths/min – Tachypneic (Normal RR for age: 14-22 breaths/min)
• Eyes: (+) periorbital edema
• Neck: supraclavicular and suprasternal retractions, (+) neck vein engorgement
• Chest& Lungs: (+) bibasal crackles
• Extremities: (+) Grade 2 bipedal edema
Lab Findings:
• CBC
o Hgb: 99 – slightly anemic
o Hct: 32 – low Hct
• Clinical chemistry
o Albumin: 18 – Hypoalbuminemia (≤ 2.5 g/dL)
o TAG: 6 (hypertriglyceridemia)
• Urinalysis
o Color: Red (hematuria)
o Protein: +++ (300-1,000 mg/dL)
o RBC: >500 (microscopic hematuria)

ADMITTING IMPRESSION
Nephrotic syndrome

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PATHOPHYSIOLOGY

Damaged visceral Increased Massive Hypoalbuminemia

epithelial cells permeability to proteinuria
(podocytes) = damaged plasma
glomerular capillary proteins
membrane
Reduced intravascular
oncotic pressure
Hyperlipidemia Increased hepatic
lipoprotein synthesis

Leakage of plasma
Activation of epithelial water into the
Proteolytic enzymes enter
sodium channels interstitium = EDEMA
tubular lumen
(ENaC)

Reduced intravascular
Increased blood volume volume

Increased aldosterone Decreased renal

Leakage of excess fluid secretion function
into the interstitium

Salt and water retention

Edema Hypertension

LEGEND:

OVERFILL HYPERLIPIDEMIA
HYPOTHESIS

UNDERFILL
HYPOTHESIS

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DIFFERENTIAL DIAGNOSIS
With the chief complaint and presence of proteinuria, we considered the following:
MOST PROBABLE DIAGNOSIS: Nephrotic syndrome
ALTERNATIVE DIAGNOSIS: Acute Nephritic Syndrome/Acute Glomerulonephritis
MUST NOT MISS DIAGNOSES: Lupus Nephritis, Membranoproliferative GN and Focal
Sclerosing Glomerulosclerosis

• Minimal Change Nephrotic Syndrome (MCNS)

Rule IN Rule OUT

✓ Edema ✗ 2-6 years old
✓ Proteinuria ✗ M>F
✓ Hyperlipidemia ✗ Presence of hematuria
✓ Hypoalbuminemia ✗ Presence of hypertension

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It was ruled out due to the following reasons:

o Our patient is 7 years of age, MCNS commonly appears between the
age of 2-6 years old
o Male predominance (our patient is female)
o The most important features of MCNS are the absence of hematuria
and hypertension.

• IgA nephropathy or Berger disease

Rule IN Rule OUT

✓ Hematuria ✗ 10-35 years of age
✓ Proteinuria ✗ M>F
✓ Edema ✗ Nephrotic syndrome presentation-
✓ Hypertension RARE
✗ No history of viral syndrome or
URTI

It was ruled out due to the following reasons:

o IgA nephropathy may present Nephrotic syndrome features such as
hyperlipidemia, hypoalbuminemia and edema but it only happens in
rare instances.
o IgA nephropathy’s onset is within 1-2 days of viral URTI

• Focal Sclerosing Glomerulosclerosis

Rule IN Rule OUT

✓ Edema ✗ 2-6 years old
✓ Proteinuria
✓ Hyperlipidemia **can’t completely rule out – will
✓ Hypoalbuminemia warrant further investigation
✓ Hypertension

• Membranoproliferative Glomerulonephritis

Rule IN Rule OUT

✓ Edema ✗ Occurs 2nd decade of life
✓ Proteinuria
✓ Hyperlipidemia **can’t completely rule out – further
✓ Hypoalbuminemia investigation needed
✓ Hematuria
✓ Hypertension

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• Lupus Nephritis

Rule IN Rule Out

✓ F>M (5:1) ✗ Adolescent period (>8 years old)
✓ Asian
✓ Hematuria **Rule out? NO. Further work-ups
✓ Proteinuria should be requested
✓ Hypertension
✓ Edema
✓ Hyperlipidemia
✓ Hypoalbuminemia

• Glomerulonephritis/Acute Nephritic Syndrome

Rule IN Rule OUT

✓ Hematuria ✗ Hypoalbuminemia
✓ Hypertension ✗ Hyperlipidemia
✓ Proteinuria
✓ Edema **Can’t be ruled out just yet because
patient has nephritic manifestations
(Hypertension, hematuria and edema)

• Nephrotic Syndrome

Rule IN Rule OUT

✓ Proteinuria
✓ Edema
✓ Hypertriglyceridemia
(Hyperlipidemia)
✓ Hypoalbuminemia
✓ Hematuria*
✓ Hypertension*

The classical hallmark of nephrotic syndrome includes:

1. Edema
2. Heavy proteinuria
3. Hypoalbuminemia
4. Hyperlipidemia

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MANAGEMENT

A. Steroids
➢ The oral corticosteroid prednisone (ex, deltason, meticorten, orasone) and
prednisolone form the mainstay of treatment for minimal change nephrotic syndrome.
o Prednisone: it is an immunosuppressant used in treatment of autoimmune
disorders. This agent may decrease inflammatory by reversing increased
capillary permeability and suppressing polymorphonuclear neutrophil (PMN)
activity.
➢ Children with onset of nephrotic syndrome between 1 to 8 year of age are likely to
have steroid-responsive minimal change nephrotic syndrome (MCNS), steroid therapy
may be initiated without renal biopsy. As per our case, our patient’s age is 7 years old
so we can start with steroids.

➢ Dosage: daily dose for 6 weeks + alternate dose for 6 weeks of steroids
➢ For steroid resistant cases ( after 8 weeks course of steroid): cyclosporine or
tacrolimus (requires renal biopsy)
o Cyclosporine and tacrolimus: They suppresses cell-mediated immune
reactions.
➢ For steroid dependent cases: need to give steroid sparing agent cyclophosphamide and
levamisole to reduced steroid toxicity.

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o Cyclophosphamide: it is cyclic polypeptide that suppresses some humoral

immune activity. In the liver, this agent is biotransformed by the cytochrome
P-450 system to its active metabolite, 4-hydroxycyclophosphamide, which
alkylates the target sites in susceptible cells in an all-or-none type reaction.
o Levamisole: it is an antiparasitic agent appears to be tied to its agnoaitic
activity towards the L-subtype nicotinic acetylcholine receptor in nematode
muscles

B. Other aspects in management

a) General
• 1st episode with mild/ moderate edema can be treated as outpatient.
• Severe symptomatic edema (effusion/ ascites) should be admitted
• Tuberculosis must be ruled out prior to starting steroid therapy.
b) Fluid balance
• Fluid restriction and low sodium diet
• Diuretics: furosemide 1mg/kg/dose IV
o Furosemide: Furosemide increase excretion of water by
interfering with the chloride-binding cotransport system,
inhibiting sodium and chloride reabsorption in the ascending
loop of Henle and the distal renal tubules.
• IV 25% human albumin 0.5g/kg
• Monitor volume status, serum electrolytes, renal function
c) Vaccination
• Pneumococcal, varicella, influenza vaccine (because there is no
history of these vaccines in our patient.)

LABORATORY TESTS TO REQUEST

1. 24-hr urine protein and Creatinine Excretion
a. Findings: Normal: <100 mg/m2/24h or <150 mg/24hr
b. Nephrotic range: 3.5 g/24h or > 40 mg/m2/hr
Change in management: still the management is same as mentioned above.
Prednisone - 60mg/m2/day or 2mg/kg/day for 4 -6 weeks Taper prednisone after 4 weeks to
alternate day therapy (starting at 40 mg/m2/day or 1.5 mg/kg/day) for 8 weeks to 5 months

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2. Spot Urine for Protein/Creatinine ratio(UPCR)

a. Findings: Normal: <0.2 mg protein/mg creatinine in >2 yrs old
b. Nephrotic range: > 2 mg protein/mg creatinine
Change in management: there is not much change in management we have to
continue Prednisone course- 60mg/m2/day or 2mg/kg/day for 4 -6 weeks and we have to
Taper prednisone after 4 weeks to alternate day therapy (starting at 40 mg/m2/day or 1.5 mg/
kg/day) for 8 weeks to 5 months
3. Renal Biopsy
a. Findings: to find out IgA nephropathy and acute renal failure
Change in management: we have to treat hypertension aggressively with renin
angiotensin blockade. A reasonable goal is a blood pressure of 130/80 mmHg. The presence of
crescents on biopsy in a sample with more than 10 glomeruli is an indication for treatment
with cyclophosphamide.
4. Electron microscopy
a. Findings: we can diagnose minimal change disease, FSGS, MPGN type 1 and 2.
There is fusion of foot process in MCD and FSGS. We can find mesangial and
subendothelial deposits in MPGN type 1 and dense deposits in type 2.
Change in management: mostly patients respond to corticosteroids.
5. Serum Complement level
a. Findings: normal in MCD and FSGS while Low level of C1,C4,C3-C9 in MPGN
type 1 and Low level of C3,C9 and normal level of C1 and C4 can be seen in
MPGN type 2.
Change in management: treat complement level accordingly after initial treatment.
6. BUN
a. Findings: elevated level may suggest renal failure
b. Change in management: Manage accordingly

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VACCINATION COMPLETE or NOT?

Based on the patient’s immunization history below, the patient’s vaccination was incomplete.
The patient is 6 years old, and she only has the following vaccinations: BCG, DPT, OPV, hepatitis B,
measles, and HIB.

Table 1. Immunization History of patient X.Y. The table shows that the patient has the following
v a c c i n a t i o n s : B C G , D P T , O P V, h e p a t i t i s B , m e a s l e s , a n d H I B .

According to the 2019 Childhood Immunization Schedule of the Philippines, the figure
below should be the guide for the list of vaccines and doses in relation to the patient’s age.

Figure 1. Immunization Schedule 2019. (Source: The Asia Parent Philippines)

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Vaccination for measles, mumps, rubella (MMR), hepatitis A and varicella should have been
started at 12 months old; Japanese encephalitis (JE) at 9 months old; and influenza at 18weeks old.
Moreover, pneumococcal conjugate vaccine (PCV) and rotavirus vaccine (RV) should have been
firstly administered at 6 weeks and 4 weeks, respectively.

References:
Villar, Anna. Bakuna 2020: Importanteng vaccines sa unang taon ni baby. The Asian Parent
Philippines. Retrieved 29 July 2020 from https://ph.theasianparent.com/bakuna.
Ramapriya Sinnakiouchenan (2020, Mar 6). Nephrotic syndrome Medescape. Retrieved 7/29/20 from
https://emedicine.medscape.com/article/244631
Kliegman, R. (2020). Nelson textbook of pediatrics (21st ed.). Philadelphia, PA: Elsevier.

Noted:

Mary Grace Padilla, MD, DPPS

Liaison Officer

Navid Roodaki, MD
Chief Resident (Clinical)

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DEPARTMENT OF PEDIATRICS
Peer Grading Sheet

Name Grades

Patel 100

Prajapati 100

Sarmiento 100

Savellano 100

Solanki 100

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