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Say-Barber-Miller syndrome

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Disease definition

A rare multiple congenital anomalies/dysmorphic syndrome characterized by microcephaly, developmental delay and intellectual disability, postnatal growth retardation, dysmorphic craniofacial features (including sloping forehead, beaked nose, large and protruding ears, micrognathia, high-arched palate, and craniosynostosis), immunologic abnormalities with transient hypogammaglobulinemia in infancy and defective chemotaxis leading to recurrent infections, as well as autoimmune/autoinflammatory phenomena. Skeletal anomalies and hypogonadism have also been reported.

ORPHA:3132

Classification level: Disorder

Synonym(s):
  • Microcephaly-hypogammaglobulinemia-abnormal immunity syndrome

Prevalence: <1 / 1 000 000

Inheritance: Unknown

Age of onset: Antenatal, Infancy, Neonatal

ICD-10: Q87.8

ICD-11: 4A01.0Y

OMIM: 251240

UMLS: C2931267

MeSH: C536618

GARD: 239

A summary on this disease is available in Français, Español, Deutsch, Italiano, Português, Nederlands, Polski
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2020.pdf) - Orphanet Urgences
Clinical practice guidelines
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.