Hereditary factor IX deficiency

Description

A disease caused by a genetically inherited X-linked recessive trait leading to a defective gene located on the X chromosome. This disease is characterised by low levels of the protein factor IX in the body leading to increased haemorrhaging and bruising due to clotting abnormalities. Confirmation is by identification of recessive trait by genetic testing.

Exclusions from above levels

JA43.3 – Postpartum coagulation defects
04 – Diseases of the immune system
05 – Endocrine, nutritional or metabolic diseases

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18 – Complications of pregnancy, childbirth or the puerperium
19 – Certain conditions originating in the perinatal period
20 – Congenital malformations, deformations or chromosomal abnormalities
22 – Injury, poisoning or certain other consequences of external causes
http://id.who.int/icd/entity/1000704511 – Human immunodeficiency virus disease
JB64.1 – Other diseases of the blood or blood-forming organs or certain disorders involving the immune mechanism complicating pregnancy, childbirth or the puerperium

Child codes

3B11.0 – Haemophilia B
3B11.Y – Other specified hereditary factor IX deficiency
3B11.Z – Hereditary factor IX deficiency, unspecified

Sibling codes

3B10 – Hereditary factor VIII deficiency
3B12 – Von Willebrand disease
3B13 – Haemophilia C
3B14 – Other inherited coagulation factor deficiency with bleeding tendency
3B15 – Inherited coagulation factor deficiency without bleeding tendency
3B1Z – Congenital or constitutional haemorrhagic condition, unspecified

Foundation entity

Hereditary factor IX deficiency – http://id.who.int/icd/entity/755266992

Part of: 03: Diseases of the blood or blood-forming organs