Holocarboxylase Synthetase Deficiency Follow-up

Updated: Apr 17, 2018
  • Author: Karl S Roth, MD; Chief Editor: Luis O Rohena, MD, PhD, FAAP, FACMG  more...
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Follow-up

Further Outpatient Care

With regular biotin administration, frequent follow-up visits to a specialist should not be required for patients with holocarboxylase synthetase (HCS) deficiency.

Growth and development should be closely observed to address residual morbidity from the initial insult.

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Inpatient & Outpatient Medications

Biotin is the only medication necessary for treatment.

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Complications

Prolonged failure to adhere to the daily biotin requirement results in an insidious onset of the underlying disease, with potentially life-threatening consequences.

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Prognosis

Early and consistent treatment allows for an excellent prognosis.

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Patient Education

Families must not become sanguine during biotin administration because affected infants appear so healthy.

Teenaged patients often rebel against taking daily medication, despite the fact that biotin is an odorless, tasteless capsule.

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