TBL 5: Facial Puffiness

By:
1. Beatrice Chia
2. Rishvinder Singh
3. Tee Ying-Yi
4. Thong Ching Fung

History
Patient demographics:
Brandon, 5 years old, Caucasian boy from Hawaii
Chief complaint: Facial puffiness for 3 days
History of presenting illness:
Patient was previously well until 2 weeks ago, he had a bad runny nose which
resolved spontaneously.
3 days ago his mother started noticing his eyes and face was slightly swollen.
The facial swelling was progressively becoming more severe thats why his
mother brought him to the hospital.
# Otherwise:
He has no other symptoms. There was no fever. No erache, sore throat,
abdominal pain, dysuria or coughing.
He did not have insect bites on his face or any parts of the body. He does not
have any known allergy. There was no trauma or burns towards his face.

Urinary:
No dysuria, hesitancy or
urgency. No polyuria or
nocturia.
Urine frequency unchanged. 3-4
times a day.
Yellow colour. No blood or
frothiness noticed in the
urine.

Systemic review:
He is still active, but slightly
lethargic than usual. No weight
gain or weight loss.
His appetite did not change.
There were no other swelling
around his body.
Respiratory:
No coughing, breathing
difficulties or noisy breathing.
CVS:
No orthopnoea, palpitations,
cyanosis
GIT:
No abdominal pain, nausea or
vomiting.No tea coloured urine,
yellowing of skin or eyes.

MSK:
There was no abnormal gait.
No muscle pain or aching.
Range of movements in joints
were optimum. There were no
joint swellings.
CNS:
He has no seizures, headaches,
confusion or drowsiness.
His vision and hearing is intact.

Antenatal/Birth history:

History
Past medical history: None.
Surgical history: none
Medication: He does not take
steroids or NSAIDs.

1. Antenatal and postpartum

uneventful.
2. SVD, at term, 3.4kg at Hawaii
Medical Centre. No NICU
admission.
3. Neonatal jaundice 3 days,
resolved spontaneously.
Feeding hx:

Allergy:
1. He has no allergy to food or
drugs
2. No allergies to
pollen/dust/pets/insect bites
3. He has no eczema, rhinitis or
conjunctivitis
Family history:
4. His family is healthy. He has an
older brother of 7 years old.
5. There was no renal diseases in
his family.
6. No
hypothyroidism/hyperthyroidism

4. Breastfed until 4 months.

Introduced formula milk.
5. Weaned at 6 months.
Supplemented adult food.
6. Current diet adult food.
Consumes meat, vegetables and
carbohydrates.
Developmental: Appropriate for
age.
Vaccinations: Up to date.

Physical examination
Vital signs:
T 37, HR 90, RR 20, BP 92/55.
Afebrile, not tachycardia, not tachypnoiec, blood pressure normal.
General Examination:
He is alert and cooperative with the examination.
His face shows moderate periorbital edema. The dorsal surfaces of
his hands and feet have mild pitting edema. He has brisk capillary
refill and 2+ pulses.
No clubbing, jaundice, palor, cyanosis or lymphadenopathy.
Hydration is good. Nutrition is adequate. No rashes are noted. His
eyes are non-injected, his conjunctiva are not edematous and his
throat is not red.
CVS: His heart sounds were regular without murmurs.
Respi: Vesicular breathing with no crackles or rhonchi.
GIT: Abdomen is soft, non-tender, non-distended and without

Lab investigations
1. Urinalysis:
Protein 4+
specific gravity of 1.030.
No hematuria

5.

Free TSH normal

6.
Liver function test normal

2. Serum electrolytes:
protein of 2 g/dL
serum albumin of 1.4 g/dL
cholesterol of 350 mg/dL.

3. BUN and creatinine - normal.

4. 24hr urine protein
collection:
albuminuria 5 g/m2/24 h

Provisional diagnosis:
idiopathic nephrotic
syndrome

Outcome
Treatment/Plan:
He is not ill enough to require hospitalization.
He is started on oral prednisone BID.
He is followed as an outpatient clinically and by daily urine
dipsticks.
Clinical course:
His edema and proteinuria gradually resolve with treatment.
His corticosteroids are tapered off and he remains stable.
Final diagnosis:
Minimal change disease responsive to steroid treatment.

Generalized vs local oedema;

Clinical Features and Differentation

Rishvinder Singh

Overview
General

Local

Cardiac

Congestive Heart
Venous
Failure
Constrictive Pericarditis

Deep Vein Thrombosis

Tumour Compression
Immobilisation,
Paralysis
Vericose Veins

Renal

Nephrotic Syndrome
Nephritic Syndrome

Lymphatic

Infections (Elephantitis)
Neoplasia
Postsurgical
Postirradiation

Hepatic

Liver Cirrhosis

Allergy

Allergens
Viral
Drugs

Endocrine

Hyperaldosteronism
Hypothyoridism

Inflammatio
n

Cellulitis
Abcess

General Oedema
General
Cardiac

Congestive Heart Failure

Constrictive Pericarditis

Increased Venous pressure. As it

worsens, renal blood supply
impairment, RAA activation,
hypoalbunimea.

Renal

Nephrotic Syndrome
Nephritic Syndrome

Hypoalbunmia
Decrease GFR, Sodium and
water retention

Hepatic

Liver Cirrhosis

Decreased protein synthesis,

hypoalbunemia.
PVH, ^ hydrostatic pressure.

Endocrine

Hyperaldosteronism
Hypothyoridism

Water and sodium retention.

(pitting)
Myxoedema (non-pitting)

Localised Oedema
Local
Venous

Deep Vein Thrombosis

Tumour Compression
Immobilisation, Paralysis
Vericose Veins

Increased pressure
intravenously

Lymphatic

Infections (Elephantitis)
Neoplasia
Postsurgical
Postirradiation

Blockage of lymphatic
return, increased
pressure intravenously

Allergy

Allergens
Viral
Drugs

Allergy leads to release

of histamine and other
factors, vasodilation

Inflammation

Cellulitis
Abcess

Vasodilation from
inflammatory disorders

Clinical Features
General

Local

Cardiac

Pedal Oedema,
Orthopneoa, Palpitations,
Giddiness, Fainting,

Venous

Pain, Tenderness, Edema,

Unilateral depending on
level.

Renal

Periorbital Oedema,
Lymphatic
Hypertension, Low urine
output, Hematuria, Frothy
urine

Primary/Congenital:
BIlateral
Secondary: Unilateral
Burning, pain, bursting
Sensitive to heat, pain,
prick

Hepatic

Ascites, Jaundice, Caput

Medusa, Purpura.

Allergy

Local pain may be

present. Abdominal pain
if intestine involved.
Sometimes, nausea,
vomiting

Collagen
Diseases

Fever, rash, joint pains.

Inflammati
on

 What diagnostic tests would you use

to help confirm the underlying cause
of edema in the child?

POST-INFECTIOUS
GLOMERULONEPHRITIS?
Investigation findings in Post-Streptococcal AGN
1. Urinalysis and culture
Haematuria present in all patients.
Proteinuria (trace to 2+, but may be in the nephrotic range;
usually associated with more severe disease.)
Red blood cell casts (pathognomonic of acute
glomerulonephritis).
Other cellular casts.
Pyuria may also be present.

2. Bacteriological and serological evidence of antecedent

streptococcal infection:
Raised ASOT ( > 200 IU/ml ).
Increased anti-DNAse B (if available) a better serological
marker of preceding streptococcal skin infection.
Throat swab or skin swab.

POST-INFECTIOUS
GLOMERULONEPHRITIS?
3. Renal function test
Blood urea, electrolytes and serum creatinine.

4. Full blood count

Anaemia (mainly dilutional).
Leucocytosis may be present.

5. Complement levels
C3 level low at onset of symptoms, normalises by 6
weeks.
C4 is usually within normal limits in post-streptococcal
AGN.

6. Ultrasound of the kidneys

Not necessary if patient has clear cut acute nephritic
syndrome.

NEPHROTIC SYNDROME
In order to establish the presence of nephrotic syndrome,
laboratory tests should confirm the existence of
(1) nephrotic-range proteinuria,
(2) hypoalbuminemia, and
(3) hyperlipidemia.

Investigations at initial presentation

1.
2.
3.
4.
5.

Full blood count

Renal profile
Urea, electrolyte, creatinine
Serum cholesterol
Liver function tests
Particularly serum albumin

6. Urinalysis, urine culture

7. Quantitative urinary protein excretion
spot urine protein: creatinine ratio or 24 hour urine protein

NEPHROTIC SYNDROME
Other investigations would depend on the
1.
2.
3.
4.
5.

age of the patient,

associated renal impairment,
hematuria,
hypertension or
features to suggest an underlying secondary cause for the nephrotic syndrome.

These tests include:

1. Antinuclear factor / anti-dsDNA to exclude SLE.
2. Serum complement (C3, C4) levels

to exclude SLE, post-infectious glomerulonephritis.

3. ASOT titres to exclude Post-streptococcal glomerulonephritis.

4. Metabolic panel - levels of serum electrolytes, calcium, phosphorus, and ionized
calcium, as well as of blood urea nitrogen (BUN) and creatinine
5. Testing for HIV
6. Testing for hepatitis B and C
7. Antinuclear antibody (ANA), antidouble-stranded DNA antibody (in selected patients)
8. Other tests as indicated

Genetic studies
Kidney ultrasonography
Chest radiography
Mantoux test
Kidney biopsy

RENAL BIOPSY ?
not needed prior to corticosteroid or cyclophosphamide
therapy.
80% of children with idiopathic nephrotic syndrome have
minimal change steroid responsive disease.
Main indication for renal biopsy is steroid resistant
nephrotic syndrome, defined as failure to achieve
remission despite 4 weeks of adequate corticosteroid
therapy.
Other indications are features that
suggest non-minimal change
nephrotic syndrome:
Persistent hypertension,
renal impairment, and/or
gross haematuria.

Management for Nephrotic Syndrome

Steroid-Sensitive Nephrotic Syndrome

Steroid-Resistant Nephrotic Syndrome

Congenital Nephrotic Syndrome

Steroid-Sensitivity Nephrotic Syndrome

85-90% of children with proteinuria will resolve with
corticosteroid therapy
These children will not progress to renal failure
Commoner in boys than in girls, in Asians than in Caucasians
Often precipitated by respiratory infections
Suggestive features:
Age between 110 years

No macroscopic
hematuria

Normal
complement
levels

Normal blood
pressure

Normal renal
function

Steroid-Sensitivity Nephrotic Syndrome

Management:
Give oral
corticosteroids (60
mg/m2 per day of
prednisolone)

Unless there are

atypical features

Median time for the

urine to become
free of protein is 11
days

After 4 weeks:
Dose is reduced to
40 mg/m2 on
alternate days for 4
weeks and then
stopped

4 + 4 = 8 weeks:
Do not respond or
have atypical
features
renal biopsy

Steroid-Resistant Nephrotic Syndrome

Management:
Should be referred to a pediatric nephrologist
Edema by diuretic therapy, salt restriction, ACE inhibitors,
NSAIDS (*also can reduce proteinuria)

Congenital Nephrotic Syndrome

Present sin the first 3 months of life

Rare
Commonest kind is recessively inherited
Associated with high mortality
(usually due to hypoalbuminaemia rather than renal failure)

Unilateral
nephrectomy may be
necessary to control
the severe
albuminaemia

Followed by dialysis
for renal failure until
the child is fit and
large enough

Renal transplant

Thank You