Pelger-Huet Anomaly [Pelgerova-Huëtova anomálie]

topical
7
Terms

Pelgerova-Huetova anomálie
pseudo-Pelgerova-Huetova anomálie
pseudo-Pelgerova-Huëtova anomálie

 

Ovoid Neutrophil Nuclei, Developmental Delay, Epilepsy and Skeletal Abnormalities
Ovoid Neutrophil Nuclei, Developmental Delay, Epilepsy, and Skeletal Abnormalities
Pelger-Huët Anomaly
Pelger-Huet Nuclear Anomaly
Pelger-Huët Nuclear Anomaly
Pseudo Pelger-Huet Anomaly
Pseudo Pelger-Huët Anomaly
Pseudo Pelger-Huet Nuclear Anomaly

Persistent link   https://www.medvik.cz/link/D010381
Definition

Autosomal dominant anomaly characterized by abnormal ovoid shape GRANULOCYTE nuclei and their clumping chromatin. Mutations in the LAMIN B receptor gene that results in reduced protein levels are associated with the disorder. Heterozygote individuals are healthy with normal granulocyte function while homozygote individuals occasionally have skeletal anomalies, developmental delay, and seizures.

Annotation
in titles & translations, use diacritic: Huët
DUI
D010381 MeSH Browser
CUI
M0016097

C Diseases
C15.378.553 Leukocyte Disorders 18
C15.378.553.231 Eosinophilia 269
C15.378.553.381 Infectious Mononucleosis 191
C15.378.553.475 Leukocytosis 97
C15.378.553.546 Leukopenia 127
C15.378.553.560 Leukostasis 7
C15.378.553.696 Pelger-Huet Anomaly 7
C16.320.051 Alagille Syndrome 19
C16.320.100 Brugada Syndrome 40
C16.320.129 CADASIL 8
C16.320.165 CHARGE Syndrome 1
C16.320.170 Cherubism 1
C16.320.180 Chromosome Disorders 259
C16.320.184 Ciliopathies 9
C16.320.188 Costello Syndrome 3
C16.320.190 Cystic Fibrosis 1 328
C16.320.215 Donohue Syndrome 1
C16.320.240 Dwarfism 97
C16.320.306 Frasier Syndrome
C16.320.314 GATA2 Deficiency 1
C16.320.365 Hemoglobinopathies 68
C16.320.467 Kallmann Syndrome 10
C16.320.480 Kartagener Syndrome 46
C16.320.488 Laminopathies 2
C16.320.540 Marfan Syndrome 105
C16.320.577 Muscular Dystrophies 140
C16.320.728 Osteochondrodysplasias 123
C16.320.784 Pelger-Huet Anomaly 7
C16.320.812 Pycnodysostosis 2
C16.320.925 Werner Syndrome 14