Table 2

What is the incidence of biotin deficiency in preschool children with global developmental delay?

StudyStudy groupStudy type (level of evidence)OutcomeKey resultComments
Sutherland et al6274 Children with low IQ, seizures, hearing loss, ataxia or motor disorder in a large outpatient clinic over a 4-year periodProspective cohort (level 4)Prevalence of BTDNone (95% CI 0 to 3.6) of the patients had BTDSample size is too small to detect BTD in the general population (95% CI 0 to 6/60 089)
Marrero- Gonzalez et al1355 patients with MR of unspecific origin born within the period 1977–1997Prospective cohort (level 4)Prevalence of BTDNone (95% CI 0 to 3.6) of the patients had evidence of BTDSample size is too small to detect BTD in general population (95% CI 0 to 6/60 089)
Zhang et al8155 children with developmental delay from various brain disordersProspective cohort (level 4)Prevalence of BTD and inborn metabolic errors11/158 (7%) had inborn metabolic errors, 1/158 had BTDPrevalence of BTD in children with MR from brain disorders is higher than in the general population
  • BTD, biotinidase deficiency; MR, mental retardation.