CONSENSUS STATEMENT:

MANAGEMENT OF IDIOPATHIC NEPHROTIC SYNDROME IN CHILDHOOD

Contents Page
Working group and workshop participants 1

1. Definition of nephrotic syndrome 3

2. Investigations at initial presentation 3

3. Management 4

3.1. Management of oedematous state 4

3.2. Management of complications of nephrotic syndrome 5

3.3. Indications for renal biopsy 6

3.4. Corticosteroids in nephrotic syndrome 6

3.4.1. At initial diagnosis 6

3.4.2. Relapse 7

3.4.3. Frequent relapses and steroid dependence 8

3.5. Cyclophosphamide 8

3.6. Relapses post cyclophosphamide 9

3.7. Urine albumin monitoring 9

4. Schema of treatment of idiopathic nephrotic syndrome 10

5. Definitions 11

6. References 12

CONSENSUS STATEMENT:
MANAGEMENT OF IDIOPATHIC NEPHROTIC SYNDROME IN CHILDHOOD

WORKING GROUP FOR INITIAL DRAFT

Adivisor : Dr. Mohd Sham Kasim

Consultant Paediatrician and Head
Department of Paediatrics
 Hospital Kuala Lumpur

Chairman: Dr. Lim Yam Ngo

Consultant Paediatric Nephrologist
Department of Paediatrics
Hospital Kuala Lumpur

Dr. Indon Lajin

Consultant Paediatric Nephrologis
Subang Jaya Medical Centre
Subang Jaya

Dr. Susan Pee

Consultant Paediatric Nephrologist
Department of Paediatrics
Hospital Sultanah Aminah
Johor Baru

Dr. Amir Hamzah

Paediatric Clinical Specialist
Department of Paediatrics
Hospital Kuala Lumpur

Dr. Lynster Liaw

Trainee in Paediatric Nephrology
Department of Paediatrics
Hospital Kuala Lumpur

WORKSHOP PARTICIPANTS

Representatives from the Ministry of Health

Dr. Wong Swee Lan Dr Pyar Kaur

Consultant Paediatrician Consultant Paediatrician
Hospital Seremban Hospital Pulau Pinang

Dr. Kwan Geok lan Dr. Soo Thian Lian

Consultant Paediatrician Consultant Paediatrician
Hospital Melaka Hospital Queen Elizabeth
Kota Kinabalu
Dr. S Tharam Dr. Irene Cheah
Consultant Paediatrician Consultant Paediatrician
Hospital Ipoh Hospital Kuala Lumpur

Dr. Leow Poy Lee Dr. Nur Khatijah Nurani

Paediatrician Paediatrician
Hospital Muar Hospital Kangar

Dr. Jamaluddin Hj Mohamad Dr. Neoh Siew Hong

Paediatrician Paediatrician
Hospital Tengku ampuan Afzan Hospital Taiping
Kuantan
Dr. Wong See Chang
Dr. Margaret Kannimmel Paediatrician
Paediatrician Hospital Sibu
Hospital Tengku Ampuan Rahimah, Kelang
Dr Tam Pui Ying
Dr. Angeline Yeoh Paediatrician
Paediatrician Hospital Sultanah Aminah
Hospital Seberang Jaya Johor Baru

Dr Kamarul Azahar Dr. Low Bin Hooi

Paediatrician Paediatrician
Hospital Seremban Tawau Hospital

Representatives from the Universities

Dr. Hans Van Rulenberghe Assoc Prof Zulkifli Ismail
Paediatric Nephrologist Department of Paediatrics
Hospital University Sains Malaysia Universiti Kebangsaan
Kubang Kerian Malaysia

Dr. Christopher CC Chua Assoc Prof Ong Lai Choo

Paediatrician/Lecturer Department of Paediatrics
University Hospital, Kuala Lumpur Universiti Kebangsaan
Malaysia

Representatives from Private Sector

Dr Gnanambai Athi Dr. Nazeli Hamzah

Consultant Paediatrician Paediatrician
Medical Specialist Centre Klinik Perdana
Air Keroh, Melaka Kota Bharu, Kelantan

Dr. David Manickam

Consultant Paediatrician
Ipoh Specialist Centre, Ipoh

CONSENSUS STATEMENT:
MANAGEMENT OF IDIOPATHIC NEPHROTIC SYNDROME IN CHILDHOOD

Idiopathic nephrotic syndrome in childhood is diagnosed by the presence of

significant proteinuria, hypoalbuminaemia and oedema, the underlying cause of
which is unknown.

Although said to be uncommon in the West at about 3 new cases per 100,000
child population, data suggests that Asians have a higher incidence at about 16
new cases per 100,000 child population . 1 Although there is no available local
data, it is felt that the incidence in Malaysia is also higher than in the West.

There are variations in the definition, investigation and management of nephrotic

syndrome in childhood. This has occasionally led to dire consequences to the
physical health of the child as well as the mental health of his/her parents. In
addition, more evidence is now available for a reasonable guideline to be
formulated.

The obvious outcome of treatment of this condition is prolonged and sustained

remission of the nephrotic syndrome with minimal side effects from treatment.

1. DEFINITION OF NEPHROTIC SYNDROME:

A clinical syndrome of massive proteinuria defined by:

1. Urine protein excretion greater than 40 mg/m 2/hour on a timed urine collection
or an early morning urine protein creatinine index of >200 mg/mmol;
2. Hypoalbuminaemia of <25 g/l,
3. Oedema.
4. Hypercholesterolaemia is not needed in definition.

It is important to ensure that there is no known primary renal disorder that has
led to the nephrotic syndrome, in particular that associated with post infectious
glomerulonephritis as the treatment for the nephrotic syndrome then depends on
the treatment of the primary renal disease.

2. INVESTIGATIONS AT INITIAL PRESENTATION

5. Full blood count,

6. Renal profile,
7. Serum albumin,
8. Urinalysis and quantification for urinary protein excretion.
9. Other investigations e.g. complement levels depends on the clinical features
and the physician in charge.

In general, the above list of investigations may suffice for children below 8 years
of age presenting with nephrotic syndrome without any other clinical features.

The International Study of Kidney Disease in Children (ISKDC) had found that at
the initial presentation of children with minimal change nephrotic syndrome -
1 20.7% of children had systolic blood pressure above 98th percentile for age;
2 22.7% had microscopic haematuria
3 32.5% had transiently raised plasma creatinine concentration
3. MANAGEMENT

3.1 MANAGEMENT OF THE OEDEMATOUS STATE.

10. Bed rest

This is not required and usually not practical unless the child has gross
oedema.

B. Diet

A normal protein diet with adequate calories is recommended. Previous

recommendations of high protein diet had not been shown to improve serum
albumin concentration.
Salt intake should be reduced during the oedematous state.

11. Antibiotics.

Children with nephrotic syndrome are more prone to primary bacterial

peritonitis. Prophylactic oral penicillin at doses of 125 mg BD or 250 mg BD
depending on the size of the child is recommended during relapse particularly
with gross oedema in view of the lack of home albuminuria monitoring and
long distance from the hospital.

Pneumococcal vaccine can be considered. However, it must be cautioned

that the vaccine does not cover all strains of pneumococci and some children
with nephrotic syndrome have been shown to be poor responders to this
vaccine.

12. Hypovolaemia.

Children with nephrotic syndrome can present with hypovolemia, the

manisfestations of which include abdominal pain, cold peripheries, poor pulse
volume, hypotension, and haemoconcentration.
The treatment is to infuse salt poor albumin at 0.5 to 1.0 g/kg/dose over one
to two hours. If salt poor albumin is not available, other volume expanders like
5% albumin, plasma protein derivatives or human plasma can be used.
13. Fluid restriction

This is not usually recommended except in chronic oedematous states.

14. Diuretics.

Diuretic therapy is not usually necessary in steroid responsive nephrotic

syndrome but if required should be used with caution as it can precipitate
hypovolemia.

Salt poor albumin of 20 - 25% concentration can be used in symptomatic

grossly oedematous states together with intravenous frusemide at 1-2 mg/kg
to produce a diuresis. There is however, the danger of fluid overload with salt
poor albumin infusion and the childs urine output and blood pressure should
be closely monitored.

15. Hypercholesterolaemia

There is insufficient evidence for a recommendation to be made as yet.