Abstract: Urea cycle disorders (UCDs) are amongst the commonest disorders
detected in critically ill newborns. True incidence of these disorders in
developing countries like India may not be known, however they are expected to
be very common owing to the high rate of consanguinity in these countries. In
this review article we discuss the problems and hardships faced by treating
clinicians in a developing country. We present an overview of the strategies to
be followed by clinicians in a developing country for treatment and management
of such disorders. Management of disorders like UCDs in a developing country
has many complexities and limitations. The outcome in these disorders depends
upon many factors like availability of basic investigations, availability of
emergency drugs, availability of special diets and their cost-effectiveness.
Alternative methods for ammonia detoxification like peritoneal dialysis and
hemodialysis may or may not be available at all the centers managing critically
ill newborns with UCDs, which may in turn affect the outcome in these children.
Liver transplants are rarely available. Thus increasing the awareness about
these disorders and educating the primary physicians in treatment and
management of such disorders become an essential part in the management of
these disorders. Emergency protocols to be followed during treatment have been
described in this review.
