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Posted On : 07 Mar 2016

May i request for advice and opinion re a clinical issue.
I am working in a secondary level hospital in South India. In the last 6 mths- we have had 3 female children presenting with a similiar clinical picture. The children are not related. All come from rural areas around the city.
The features are as follows.
Female child- age 2-5yrs.In good health prior to onset of symptoms.
Presents with a h, o an antecedent resp. tract infection a few days prior to admission. At admission- h, o seizures, and difficulty in breathing.
Clinical examination- child in marked respiratory distress. with acidotic breathing. Poor peripheral perfusion.
Conciousness obtunded - with GCS- 5-8
Hepatomegaly.
No cardiac , respiratory signs.
A working diagnosis of Septic Shock with metabolic acidosis made` Reye`s syndrome also kept in mind. treatment started accordingly.
Investigation results - the only remarkable feature is
persisting metabolic acidosis. The metabolic acidosis is difficult to correct despite large infusions of Sodium Bicarbonate and ventilation.
Despite intensive care and ventilation- none of these children have survived.
repeated blood gas while on the ventilator shows metabolic acisosis.
There is no history of ingestion of any toxic substance.
Could anyone suggest some possibilites_? Thank You.

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Answer 1

Rule out inborn errors of metabolism.

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