Severe congenital neutropenia (SCN), also often known as Kostmann syndrome or disease, is a group of rare disorders that affect myelopoiesis, causing a congenital form of neutropenia, usually without other physical malformations. SCN manifests in infancy with life-threatening bacterial infections.Most cases of SCN respond to treatment with granulocyte colony-stimulating factor (filgrastim), which increases the neutrophil count and decreases the severity and frequency of infections. Although this treatment has significantly improved survival, people with SCN are at risk of long-term complications such as hematopoietic clonal disorders (myelodysplastic syndrome, acute myeloid leukemia). Kostmann disease (SCN3), the initial subtype recognized, was clinically described in 1956. This type has an autosomal recessive inheritance pattern, whereas the most common subtype, SCN1, shows autosomal dominant inheritance. Presentation
Infants with SCN have frequent infections: 50% have a significant infection within 1 month, most others by 6 months. Their etiology is usually bacterial, especially staphylococcal, and they commonly involve abscesses, both cutaneous and of internal organs, pneumonia, mastoiditis (inflammation of the mastoid process), and sepsis. All of these are life-threatening for infants. Genetics
Kostmann disease, SCN3, is inherited in an autosomal recessive manner, but the commonest subtype of Kostmann syndrome, SCN1, is autosomal dominant.A significant proportion of SCN lacks a known mutation. The recognized subtypes of Kostmann syndrome are:

SCN1 is the commonest form of SCN, which accounts for 60-80% of SCN, and the first to be genetically typified. This autosomal dominant form that arises from mutations of the ELANE (formerly ELA2) gene on chromosome 19p13.3, which encodes neutrophil elastase.
Clinodactyly
Clinodactyly is a medical term describing the curvature of a digit (a finger or toe) in the plane of the palm, most commonly the fifth finger (the "little finger") towards the adjacent fourth finger (the "ring finger"). It is a fairly common isolated anomaly which often goes unnoticed, but also occurs in combination with other abnormalities in certain genetic syndromes, such as Down syndrome, Turner syndrome and Cornelia de Lange syndrome. In birds
Anisodactyly
Anisodactyly is the most common arrangement of digits in birds, with three toes forward and one back. This is common in songbirds  and other perching birds, as well as hunting birds like eagles, hawks, and falcons. This arrangement of digits help with perching and/or climbing and clinging. This occurs in Passeriformes, Columbiformes, Falconiformes, Accipitriformes, Galliformes and a vast majority of other birds. Syndactyly
Syndactyly, as it occurs in birds, is like anisodactyly, except that the third and fourth toes (the outer and middle forward-pointing toes), or three toes, are fused together, as in the belted kingfisher (Megaceryle alcyon). This is characteristic of Coraciiformes (kingfishers, bee-eaters, rollers, and relatives). Zygodactyly
Zygodactyly (from Greek ζυγος, even-numbered) is an arrangement of digits in birds and chameleons, with two toes facing forward (digits 2 and 3) and two back (digits 1 and 4). This arrangement is most common in arboreal species, particularly those that climb tree trunks or clamber through foliage. Zygodactyly occurs in the parrots, woodpeckers (including flickers), cuckoos (including roadrunners), and some owls.