IEM Handbooks and Guidelines

IEM Dietary Handbooks

The Australasian Society for Inborn Errors of Metabolism have produced a number of handbooks to aid dietitians and families in the treatment of metabolic disorders.

These handbooks can also be found on their website

ASIEM Galactosaemia Handbook

ASIEM Homocysinuria Handbook

ASIEM Hyperornithinaemia Handbook

ASIEM Maple Syrup Urine Disease (MSUD) Handbook

ASIEM Organic Acidurias Handbook

ASIEM Phenylketonuria (PKU) Handbook

ASIEM Tyrosinaemia Handbook

ASIEM Urea Cycle


National PKU Guidelines

(ASIEM Endorsed)

Announcing New National Guidelines for PKU Management in Australia.

In 2013 MDDA asked ASIEM to consider working on consistent national treatment guidelines.  over the past few years we have received a standard protein counting as well as guidelines for specific clinical groups (e.g. maternal women). We pleased that ASIEM has now announced the release of treatment guidelines for PKU across the lifespan.  These guidelines are based on both evidence where it is available and clinical experience.  The guidelines have been developed with input from metabolic teams across Australasia.   The MDDA sees the guidelines as important tool for us as patients.  They can help us ask informed questions about our treatment and discuss our options with clinicians.  They are guidelines and each family or individual should work with their metabolic team to find the best fit for their individual situation.

If you have any questions about any aspect of the guideline please free free to contact the office.

Australasian PKU Lifespan Guidelines

National PKU Maternal Guidelines

National PKU Protein Counting Guidelines

Australasian BH4 Guidelines

This booklet provides a general overview of the Low Protein Diet recommended for people with PKU, as well as instructions and tables for protein counting.

PLEASE NOTE: This information should be used in conjunction with tailored dietary advice from your specialist Metabolic Dietitian.

Anita Inwood (ASIEM Chair)