intestinal pseudoobstruction, neuronal, chronic idiopathic, X-linked
Intestinal pseudo-obstruction is a condition characterized by impairment of the muscle contractions that move food through the digestive tract. The condition may arise from abnormalities of the gastrointestinal muscles themselves (myogenic) or from problems with the nerves that control the muscle contractions (neurogenic). When intestinal pseudo-obstruction occurs by itself, it is called primary or idiopathic (unknown cause) intestinal pseudo-obstruction. The disorder can also develop as a complication of another medical condition; in these cases, it is called secondary intestinal pseudo-obstruction. Individuals with this condition have symptoms that resemble those of an intestinal blockage (obstruction) but without any obstruction. It may be acute or chronic and is characterized by the presence of dilation of the bowel on imaging. The causes may be unknown or due to alterations (mutations) in the FLNA gene, other genes or are secondary to other conditions. It may be inherited in some cases. Intestinal pseudoobstruction neuronal chronic idiopathic X-linked is caused by alterations (mutations) in the FLNA gene which is located in the X chromosome. There is no specific treatment but several medications and procedures may be used to treat the symptoms.
MONDO:0003847
hereditary disease
is_a
MONDO:0014097
congenital short bowel syndrome
is_a
MONDO:0017574
chronic intestinal pseudoobstruction
is_a
MONDO:0700007
idiopathic disease
is_a
CIIP
related
CIIP X-linked
related
CIIPX
related
congenital short bowel syndrome, X-linked recessive
exact
intestinal pseudoobstruction neuronal chronic idiopathic X-linked
related
intestinal pseudoobstruction, neuronal, X-linked recessive
exact
intestinal pseudoobstruction, neuronal, chronic idiopathic, X-linked
exact