Code System Concept

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Code System Concept

Code System Concept Code	234161007
Code System Concept Name	Familial pulmonary capillary hemangiomatosis (disorder)
Code System Preferred Concept Name	Familial pulmonary capillary hemangiomatosis (disorder)
Concept Status	Published
Concept Status Date	09/01/2024
Code System Name	SNOMED-CT

Concept Relationships
Concept Details

Concept Details

Code System Code	PH_SNOMED-CT
Code System OID	2.16.840.1.113883.6.96
HL7 Table 0396 Code	SCT
Superseded By Concept
SDO Concept Status	Active
SDO Concept Status Date	01/31/2002
SDO Date Created	01/31/2002
SDO Date Revised	09/01/2023
Description	An autosomal recessive subtype of primary pulmonary hypertension which has histological characteristics of widespread fibrous intimal proliferation of septal veins and preseptal venules. There is frequent association with pulmonary capillary dilatation and proliferation and the disease can cause occult alveolar hemorrhage.
Sequence

Extended Properties

No extended properties.

Concept Alternate Designations

SDO Designation ID	Concept Designation Description	Status	Designation Type	PHIN Preferred Term Flag	Code Flag
350829012	Familial pulmonary capillary haemangiomatosis	Active	Synonym	false	false
350830019	Familial pulmonary capillary hemangiomatosis	Active	Synonym	false	false

Associated Value Sets

Value Set Name	Version(s)
Clinical Finding (NNDSS)	2 , 1
Disease	10 , 9 , 8 , 7 , 6 , 5 , 4 , 3 , 2 , 1
Problem Value Set	3 , 2

Parent Concepts

Parent Concepts

Parent/Child (Relationship Type)

Autosomal recessive hereditary disorder (disorder) {85995004 , SNOMED-CT } Cardiovascular system hereditary disorder (disorder) {363005004 , SNOMED-CT } Congenital anomaly of trunk (disorder) {78626001 , SNOMED-CT } Disorder of capillaries (disorder) {58729003 , SNOMED-CT } Disorder of thorax (disorder) {118946009 , SNOMED-CT } Familial disease (disorder) {111941005 , SNOMED-CT } Pulmonary capillary hemangiomatosis (disorder) {233949008 , SNOMED-CT } Specific mixed vascular syndrome (disorder) {234158006 , SNOMED-CT }

Other Relationships

No other relationships present.

Child Concepts

No child concepts present.

Page last reviewed:June 20, 2024
Page last updated:June 20, 2024
Content source:
- Centers for Disease Control and Prevention,