Autosomal recessive hereditary disorder	Brachydactyly	Cardiovascular system hereditary disorder	Chondrocalcinosis	Congenital anomaly of cartilage	Congenital peripheral pulmonary artery stenosis	Connective tissue hereditary disorder	Developmental hereditary disorder	Hereditary disorder of musculoskeletal system	Multiple malformation syndrome with facial-limb defects as major feature
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Keutel syndrome (disorder)
	Keutel syndrome
	Pulmonic stenosis, brachytelephalangism, calcification of cartilage syndrome
	A rare multiple congenital anomalies/dysmorphic syndrome characterized by diffuse cartilage calcification, brachytelephalangism, peripheral pulmonary artery stenoses and facial dysmorphism. Vascular calcification has been reported in some cases.

Id	724208006
Status	Primitive

Associated morphology	Stenosis
Finding site	Pulmonary artery within lung
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Pathologic calcification
Finding site	Cartilage structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Morphologically abnormal structure
Finding site	Face structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Abnormally short growth
Finding site	Digit structure
Occurrence	Congenital
Pathological process	Pathological developmental process

SNOMED CT to Orphanet simple map

SNOMED CT to ICD-10 extended map

Target	Q87.8
Rule	TRUE
Advice	ALWAYS Q87.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified